1. health and fitness
2. disease

SOUVENIR
WOMEN CARDIAC CARE & INTERVENTIONAL UPDATES
2015
(WCC&IVUS2015)
DATE: 7th & 8th MARCH, 2015
NIZAM'S INSTITUTE OF MEDICAL SCIENCES
HYDERABAD
Prof. Damera Seshagiri Rao
(President)
Prof. Maddury Jyotsna
(Organizing Secretary)
Inaugurated
By
Eminent Women Cardiologist
Prof. Savitri Srivastava
Director,
Fortis Escorts Heart Institute & Research Centre,
New Delhi
PROF.L.NARENDRA NATH
DIRECTOR, NIMS
Dear All,
We are pleased to inform you about “WOMEN CARDIAC CARE & INTERVENTIONAL
UPDATES 2015(WCC&IVUS2015)” conference on 7th and 8th March 2015 by Dept of
Cardiology at Nizam’s Institute of Medical Sciences (NIMS) on the eve of “world
women’s day”.
Theme for International Women's Day 2015 is “Empowering Women - Empowering
Humanity: Picture It!”. Let us try to empower all women so that whole humanity gets
the strength.
The program is especially for women of all ages. Whatever is the woman’s age, she
needs to take action to protect her heart health. Heart disease can begin early, even in
the teen years, and women in their 20s and 30s need to take action to reduce their risk
of developing heart disease. Pregnancy related heart diseases require special attention.
Older women also require attention as it's never too late to take action to prevent and
control the risk factors for heart disease, especially degenerative aortic valve diseases.
Even those who have heart disease can improve their heart health and quality of life.
Nearly 40% of our cardiac interventions were in female patients, we require special
attention as the complication rates are more in females. So, this time onwards we
added interventional component also to WCC. We will have live case transmissions
from International and National Institutions and complex case presentations covering
various topics of interest. Our primary care and secondary care physicians and
cardiologists who participate in care of many of these patients will be updated through
advancement of knowledge and evidence based guidelines through a special track. This
conference will bring together all experts, scientists and postgraduate students from
the field of cardiology to a single platform and will provide opportunities to exchange
ideas and experiences with the pioneers and experts in their respective fields.WCC &
IVUS2015 will include Guest lectures, CME and live cases along with oral
presentations.
We are welcoming you to WOMEN CARDIAC CARE & INTERVENTIONAL UPDATES
2015(WCC&IVUS2015) on 7th and 8th March 2015 at Nizam’s Institute of Medical
Sciences (NIMS).
Yours sincerely,
Prof. Maddury Jyotsna
Organizing Secretary
Cardiovascular diseases have surpassed malignancy and emerged as the major killer
in women. Hence, there is a need to focus our attention on women’s cardiac care.
Though it is a general belief in the society that heart diseases are mostly confined to
men, the data shows the women are affected in increasing numbers. Women account
for 1/6th of the global burden of heart diseases. To re-emphasize the need for greater
attention for women cardiac care, the theme of the World Heart Day 2013 was
dedicated to women and children.
The symptoms in women are often atypical, as a result, they do not seek medical
attention promptly. First episode of attack in women carries greater mortality than
men. This highlights the importance of the family Physician and also Obstetrician /
Gynecologist to screen their patients for heart diseases and refer them promptly to the
Cardiologists. The role of woman is central to the health of entire family. They should,
not only keep up their cardiac health, but also influence the health care of the
children, husband and other elders in the family by inculcating good dietary habits,
exercise and reduction of stress.
In continuation of our commitment to women’s cardiac care, the Department of
Cardiology, NIMS, is organizing a workshop on 7th & 8th March, 2015 to rededicate
ourselves to the cause of women cardiac care. This meeting will have lot of interactive
sessions among the various Specialists, Cardiologists, Gynaecologists, Imageologists,
Cardio-thoracic Surgeons for sharing their knowledge.
Dr. D. SESHAGIRI RAO
President
PROF. & HOD
DEPT. OF CARDIOLOGY
MESSAGE
I am happy to note that the Department of Cardiology is organizing “Women Cardiac Care &
Interventional updates 2015” on 7th and 8th March 2015.
The scientific programme is focused on Cardiac Diseases of women from paediatric to geriatric age.
Discussion on complex cases and live demonstration of interventional procedures facilitate teaching and
learning from experts.
I congratulate Prof. M. Jyotsna for all her efforts in organizing this programme.
I wish the CME a great success.
DEAN
Prof. C. Sundaram
Women in Cardiology- Challenges and Opportunities
Congratulations to all women in Cardiology – we have come a long way!
Despite the long history of women providing medical care over centuries, the first
woman to be formally trained in medicine was not until the end of 19th century.
Women were not allowed entry into medical schools as they were considered to be
physically and intellectually inferior. It was thanks to the courage of pioneers like
Elizabeth Blackwell, Rebecca Lee Crumpler and Elizabeth Garrett that women emerged
as qualified doctors. In fact one of the women pioneers, D r Margaret Buckley had to
dress as a man to gain admission to medical school in UK in the19th century.
Considering that the first woman cardiologist was not trained till 1960s in Switzerland,
one can appreciate the long journey women had to undertake to be accepted as
cardiologists. Even in the 21st century, the statistics show that less than 25% of
cardiologists were women and less than a third of these are interventionists. If you
take the fact that nearly 60% of women admitted to medical schools are women, these
figures further highlight the discrepancies.
Obstetrics and Paediatrics were the only specialities thought to be ‘suitable’ for women
until the mid 20th century, especially in India. Even general medicine was thought to
be a departure from the norm. Paradoxically, Dr Padmavathy established herself as
Cardiologist in Delhi in early 1950s, which is no mean feat.
The greatest obstacle for women to take up cardiology is, in my opinion, this
stereotyping itself. It is easy to accept the norm and go with the flow, which is less
stressful and probably more ‘successful’. However, if you define success as achieving
ones full potential, then perhaps women doctors are the ones who compromise the
most.
Quite often, woman’s role as homemaker is argued as the reason for not entering a
male dominated specialty. But in truth, is it not more onerous to be an Obstetrician
with emergencies which are unpredictable and which cannot be hurried?
Having to work unsociable hours is again not unique to Cardiology and probably more
so in the ‘traditional’ specialities alike.
Working in a male dominated environment is probably what discourages women most.
If you take a look around you, actually you will find that apart from the doctors, most
of the staff on your wards or catheterization laboratory are women – nurses,
technicians, radiographers etc
Exposure to radiology is another commonly perceived hurdle. But with modern
radiation protection techniques, the risk of radiation exposure is minimal and equal to
both genders.
Of course, a woman has to avoid radiation during pregnancy, and it is not true that
the absences from the catheterization laboratory due to maternity can ‘deskill’ the
doctor. You only have to look at the leading Interventionists in India to realize that
many of them had no access to cath lab for years, between the time of training and
starting the interventions.
An important obstacle remains the acceptance by the patients and public of a woman
cardiologist, especially an Interventionist. From personal experience, I feel that the
patients accept a competent doctor, regardless of their specialty and gender. It is true
that there may be some initial resistance from the public and the opportunity to prove
ones worth may be difficult to find. That is probably why more women opt to work in
an Institute rather than foray into individual practice as cardiologists.
The greatest obstacle is however the attitude of male colleagues, often patronizing,
frequently demeaning, towards their female equivalents. They often treat women as
intellectual inferiors, expect them to be subservient and take credit for their
achievements. But it is important to realize that it is not only women cardiologists
who are exposed to this kind of behaviour. It is true of any member of a minority
group who is posing a challenge to their equals. As with any situation leading to
harrassment, it is important to remain focused, keep calm and carry on!
Having said that, a man or woman who is confident of their own stature, does not
discourage a fellow human being from developing. It is owing to these men of vision
that women had been able to break the barriers and prove themselves.
Is it important that women should become cardiologists? Let us see…
Cardiac disease is in women is more challenging not only in terms of diagnosis but
also in terms of treatment. Many randomized trials in cardiology had under
representation of female patients and the lessons learnt from predominantly male
Caucasian population were applied to the treatment of Indian women.
The unique physiological and pathophysiological features were not taken into
consideration in evaluating the symptoms in women and generalizations were made
that ‘women had atypical symptoms and difficult to diagnose’– judgments made mostly
by male physicians!
It was indeed not until the 1970s, that there were clinical trials specifically looking at
heart disease in women. It is interesting that the formal training of women in
cardiology started in 1960s, which I am sure has provided the impetus for the
attention in cardiological problems in women.
It is not only beneficial for the female patients, but the caring and compassionate
attitude of women doctors is appreciated by all patients, men and women alike.
It is time that women take their rightful place in all fields of society – not only
cardiology but surgery etc.; not only medical field but academical, political and
leadership too. One should not interpret it as feminism to uphold basic human values
and equality. We do however, need infrastructure to encourage women to fulfill their
potential – not by positive discrimination but by providing equal opportunities and
environment conducive to their development.
Lastly, it is up to women themselves to believe in their capabilities and demonstrate
their worth.
“…But I have promises to keep, and miles to go before I sleep and miles to go before I
sleep” - Robert Frost
-
Dr Rama Bala
Consultant Cardiologist
Kings College NHS Trust, London
LIVE CASE COURSE DIRECTORS
Prof. Ran Kornowski
Director of the Institute of Interventional Cardiology
Rabin Medical Center (Beilinson and Hasharon)
Israel
DR. KIRTI PUNAMIA, MUMBI, INDIA
PROF. D. SESHAGRIRI RAO, NIMS,INDIA
PROF. M. JYOTSNA , NIMS, INDIA
PROF. SAI SATISH, NIMS , INDIA
PROF. LSR KRISHNA, NIMS, INDIA
SELECTED ABSTRACTS FOR ORAL PRESENTATIONS:
SESSION: PREGANANCY AND HEART DISEASE
1. Acute Pulmonary Edema Secondary to Cardiac Failure – Non Compacted Left
Ventricle in Pregnancy - Dr.Anisha Gala
Case summary
Mrs MS, 22yrs, Primi @ 35+6 weeks, Diagnosed as preterm labour - Tab Nifedepin
20mgTid + Inj Betamethasone 12 mg IM stat. Shifted to FH @ 00.15 on 15th October
2013
State of CV collapse, Non palpable peripheral pulses, Bradycardia (30 bpm),
Tachypnoea (RR 50/min) SpO2: 77% , O2 via mask. Asystole within seconds of arrival
to FH. Immediate resuscitation done. CPR / ACLS / ETT / IPPV . Perimortem CS in
HDU @ 00.20 AM. Alive male baby, 2.12 kg, Apgar 3/6/8. ROSC in 4 minutes.
Extreme persistent tachycardia @ 180/min . ICU care & Ventilatory Care, Cardiac
Monitoring. IV antibiotics / IV Frusemide / LMWH, Other Supportive measures
Investigations : CBC, CUE, LFT, RFT , coagulation profile were normal. 12 Lead ECG
- Sinus Tachycardia, Inverted T waves ( Rate related ). X-ray Chest : Cardiomegaly
2D Echo : Dilated LV / Global Hypokinesia, Severe LV systolic dysfunction , Ejection
Fraction : 26%. Mild MR / TR ……… No Thrombus / Embolus, ? Non Compacted LV
8th & 9th Post Operative day - Shifted to ward, Repeat 2D Echo – EF of 48%, NCLV,
Discharged on Tab Ramipril - 5mg/OD, Tab Carvedilol - 3.125mg / BD , Tab
Torsemide 10 mg / OD x 10 days. Review with Physician after 10 days. Review with
Cardiologist after 2 weeks. Repeat 2DE after 3m – confirmed NCLV
Cardiomyopathies and Pregnancy : Cardiomyopathies are rare diseases but may
cause severe complications in pregnancy. Etiology of cardiomyopathy in pregnancy Peripartum Cardiomyopathy (PPCM), Hypertrophic Cardiomyopathy (HCM), Dilated
Cardiomyopathy (DCM), Restrictive Cardiomyopathy (RCM), Arrythmogenic RV
Cardiomyopathy (ARVC), Unclassified Cardiomyopathies (LV Non compaction
Cardiomyopathy (LVNC), Alcoholic, Viral, Stress cardiomyopathy, Idiopathic
cardiomyopathy
Non compacted LV ( LVNC ) : PREVALENCE - Very low incidence, 1 in 2000
echocardiographic studies. Awareness resulting in increased reporting. Over / under
reporting 2nd diagnostic difficulties
Apical 4-chamber view, showing hyper-trabeculation of the mid-apical segments of
the lateral
wall.
•
Colour Doppler showing blood flow within the trabecular recesses of the apex.
•
2004-06 National survey in France: 154 cases reported as LVNC. 105 cases
reconfirmed as LVNC, 49 cases the diagnosis was questionable. Cases followed for
2.33 years -Common circumstances leading to the Diagnosis, CCF in 50%, Evaluation
of DCM, Rhythm abnormality, Embolic Events. Familial screening in 8% .Age Range:
18 – 86 years, Male : Female = 66% : 34%.
Congestive Cardiac Failure - 30% - Recurrent admissions for CCF, 4% - Cardiogenic
Shock, 9% - Cardiac transplant, 4% - waiting on transplant list. Other complications
were Rhythm abnormalities, Embolic Events with high Mortality
Literature review : Paucity of cases reported with LVNC & pregnancy (9 so far). All
suggest – The management of pregnant patients with any inherited cardiomyopathy is
directed to the usual treatment of heart failure with diuretics (with or without digoxin)
or alternatively hydralazine and nitrates. Early delivery due to heart failure is frequent
Conclusion : High index of suspicion to diagnose LVNC. Pre-pregnancy assessment .
Standard heart failure treatment with ACE and aldosterone inhibition together with
diuretics and beta-blockers post-delivery is likely to promote recovery of ventricular
function once the baby is delivered. Lactation is an additional stressor which may
exacerbate heart failure post-delivery . Concerted efforts of the team of Obstetrician,
Cardiologist, Anesthetist, Neonatologist are mandatory to ensure optimal Outcome.
2. Incomplete Lupus and Complete Heart Block in Pregnancy: A Case Report - Dr.
Anjani Kiranmayi
Introduction:
Incomplete lupus is defined as the presence of fewer than four of the ACR criteria for
SLE. The prevalence of conduction defects in SLE may be as high as 10-14%. Third
degree AV block is very rare in adults with SLE.
Pregnancy complicated with complete heart block is rare and a high risk
condition. Permanent cardiac pacing is indicated in symptomatic cases in second
trimester. The risk of permanent pacemaker implantation is low.
CASE REPORT:
A 23 year old pregnant female, primi of 28 weeks gestational age came with the
complaints of giddiness since 2 months with syncopal episodes. There was no past
history of heart disease. There was no family history of heart disease.
On examination her pulse rate was 36 per minute and BP was 90/60 mm Hg.
Her ECG showed complete heart block with AV dissociation with a junctional
escape rate of 36 per minute. Her 2D echo showed no evidence of structural
abnormality with normal LV function.
She tested positive for ANA with 3+ intensity with speckled pattern and her anti dsDNA was positive with levels of 1.7.
ECG showing CHB with AV dissociation:
Rhythm strip showing CHB:
Her fetal echo showed no evidence of congenital heart disease or complete heart
block.
As the patient was symptomatic, she was implanted with a temporary pacemaker
followed by permanent. Since then, she was asymptomatic.
ECG after pacemaker implantation:
3.Safe motherhood by interventions during pregnancy with cardiac valvular
disease - Dr.Mupparapu Laxmi Prasanna
Introduction
Mordern science has improved so much that early diagnosis & treatment and
intervetions during pregnancies also has resulted in successful & fruitful maternal
&perinatal outcomes. Here are the two case reports from Gandhi Hospital- pregnancy
with severe aortic stenosis & second case with mitral valve stenosis with interventions
in fifth month
Case 1 : Mrs.X., 26 YRS Primigravida with 36-37 weeks admitted for institutional
delivery. Known case of subaortic membrane operated in childhood @ 10yrs age at
NIMS Hospital-aortic commisurotomy –SAM resection. She presented with dyspnoea
grade 2 in 5months of pregnancy for which PBAV(percutaneous ballon aortic
valvuloplasty was done on 14/6/2014 for severe aortic stenosis at Mediciti hospitals.
Family history – nil significant. On examination –GC fair ,vitals stable, all peripheral
pulses felt, Heart-S1 S2+, systollic murmur +, lungs clear. P/A-uterus 37 weeks,
relaxed, cephalic presentation, liquor adequate, FHR good. Patient not in labour.
Investigations : Hb%-9.8gms, RBS-98gms/dl, Blood urea-20mgs, S.creatinine-0.7. 2D
ECHO-EF-77%,s/o PBAV,mod.A.S,mild AR,mild MR,good LV function, mild TR & mild
PAH. USG-SLF with 37 weeks gestation,placent posterior upper segment grade 2,liquor
adequate.
Patient had PROM on 30/9/2014 at 9.30pm, IE prophylaxis was given. Given birth to
a live term female baby on 1/10/2014 at 4.30 am , 2.5kgs, good apgar, vaginally.
Intrapartum was uneventful. On 2nd postnatal day she had lower respiratory tract
infection, subsided with antibiotics. Patient was discharged on 9th postnatal day.
Case 2 : Mrs.S, 30yrs, G2P1L1 with 36weeks pregnancy, previous section, CRHD,
BREECH PRESENTATION, LMP-26/5/2013, EDD-3/3/2014. She presented with
dyspnoea grade 1 in second trimester, for which she was investigated and diagnosed
as mod. Mitral valve stenosis. After a week she underwent PBMV at private hospital
in nov. 2013.
Obstetrics history- 1st -concieved spontaneously. Had c-section & delivered an alive
term female. 2nd-conceived after 9yrs .In second trimester she presented with SOB
grade 2 & was diagnosed as moderate mitral valve stenosis. Had regular cardiology
checkups at gandhi hospital & on penidure injection. At 7th month she was started on
lasix tablet 10mg od, syrup potchlor. At 9th month cardiologist added tab.Aten 25mgonce daily.
On examination –GC fair , vitals stable,JVP-normal,Heart-S1S2+,MDM+,Lungs-clear.
P/A-uterus-36weeks,breech presentation, relaxed, liquor adequate, supra-pubic
transverse scar +. Patient not in labour.
Investigations : Hb%-10gms. RBS-100mgs%. Blood urea-20mgs. S.creatinine-0.7. LFTnormal. ECG-normal. 2D ECHO-(13/1/2014)-EF-60%,CRHD,mod MS, mild MR,trivial
TR/ no PAH, good RV & LV function.
Posted for elective c-section under epidural anaesthesia – IE prophylaxis was given,
delivered an alive term female neonate 3kgs,on 15/2/2014. Intraop & postoperative
period was uneventful. Discharged on 10th postoperative day.
SESSION: CONGENITAL HEART DISEASE & PAH
1.Anomalous Origin of Coronary Arteries from Pulmonary Artery presented in 6th
decade - Therapeutic dilemma.- Dr.J V Pradeep kumar
Anomalous origin of coronary arteries from pulmonary artery usually present in early
childhood and it is very rare to survive till 6th decade of life. We are presenting 2 case
reports which presented to us in 6th decade and gave us therapeutic dilemma- should
we do surgery or leave on medical management?
1st case: 63 year female presented in the emergency with an acute episode of dyspnea,
hypotension and transient loss of conscious. On admission she was intubated and
started with inotropes. ECG showed ST depression in V2- V4 with strongly positive for
Troponin. 2D Echo showed RWMA in LCX territory with moderate LV dysfunction.
After she was stabilized coronary angiogram was done which showed dominant large
right coronary artery which was filling the entire LAD system retrograde. CT coronary
angiogram showed origin of left coronary artery from the main pulmonary artery.
Before discharge, her LV function was normal.
2nd case: 70 year old lady, well-built presented with class II angina. Her coronary
amigo showed anomalous origin of right coronary artery from pulmonary artery.
Patient undergone CABG with good outcome.
2.Safe motherhood in a third gravida with ebstein’s anomaly and severe preeclampsia - Dr. K Anusha
Introduction
Ebstein’s anomaly is a rare congenital cardiac malformation characterized by apical
displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization
of the right ventricle with a variable degree of malformation and displacement of the
anterior leaflet.
Our case is one of the rare cases of a woman with Ebstein’s anomaly with three
successful vaginal deliveries.
Case details
Mrs. G, 30 yrs female, Gravida3 Para2 Live2, Term gestation, known case of Ebsteine’s
anomaly , diagnosed during her first pregnancy. Referred from outside in view of
anemia and severe pre eclampsia. On admission patient was asymptomatic. No
history of breathlessness, chest pain, palpitation, syncopal attacks or cyanotic spells.
No symptoms suggestive of imminent eclampsia
Diagnosed as having severe pre eclamsia at 8months of pregnancy on medication.
Diagnosed as having Ebsteins anomaly during first pregnancy 6yrs back coincidentally
during antenatal checkups. She has no cardiac symptoms at that time
Obstetric History : First pregnancy FTNVD, female ,healthy, 2.5kg. 2nd pregnancy
FTNVD, male, healthy, 3kg. No congenital heart disease was diagnosed in them. She
has history of pre eclamsia in first pregnancy . Intrapartum and postpartum period
uneventful.
General examination: Conscious and coherent, Pallor +, Pedal edema grade 2, PR
102/min, regular, rhythmic, normal in volume all peripheral pulses felt . Respiratory
Rate 22/min. BP 200/120mmHg in right arm on admission. CVS: systolic murmur
grade 1 in mitral and tricuspid area. Lungs : vesicular breath sounds heard. No added
sounds
Obstetric examination: Obstetric examination revealed a term gestation in
cephalic presentation with clinically less liquor a good fetal heart rate and relaxed
uterus. Pelvic examination revealed that patient was not in labour.
Investigations : Hb- 11.7gm%, Platelets-1.8lakhs/ cumm, RBS-80mg/dl, RFT &LFT
normal, Urine albumin 2+, Obstetric USG- Single life fetus , 32 Weeks gestation,
cephalic presentation,
amniotic fluid index of 5 , grade 2 placental maturity .
ECG Normal. 2 D Echo – Ejection fraction 78%, Tricuspid valve : septal leaflet apically
displaced
severe tricuspid regurgitation, mild PAH, dilated right and left atrium, good left and
right ventricular function.
Patient was hospitalized. Her hypertension was under control with iv labetalol.
Cardiologist categorized the patient as NYHA functional class 1- low risk for vaginal
delivery or cesarean section. She delivered vaginally term female baby of 3.2 kgs with
good apgar score spontaneously after 4 weeks . She had mild PPH which was
controlled by uterine massage and uterotonics. She was observed in ICU for 24
hours. Rest of post partum period uneventful. Albuminuria resolved within 10 days.
Hypertension was controlled with metoprolol. Discharge after 2 weeks. No congenital
heart disease was detected in baby
3. Single coronary ostium in the right aortic sinus - Dr.Palaparthi Raghuram
Anomalous origin of coronary arteries represents a clinical challenge because of the
anatomical variability and possible functional consequences, the pathophysiological
mechanisms involved, and the lack of large published series that would provide
evidence to guide the clinical and therapeutic approach. We report a 48 yr old diabetic
female who presented with chest pain, palpitations and fatigue for 6 months. Her
electrocardiogram, 2D echocardiographic examination, stress testing were normal.
Coronary angiogram was done in view of persistent symptoms and diabetes which
showed single coronary ostium in the right aortic sinus with anomalous origin of LAD
from proximal superdominant RCA with absent LCX. Epicardial coronaries were free of
atherosclerotic disease. CT coronary angiogram was done to evaluate the course of
anomalous LAD, which showed prepulmonic course of LAD excluding any extrinsic
compression. What makes this case unusual is that, single coronary ostium without
other major cardiovascular abnormalities in itself is a rare entity. To our knowledge,
the anatomical subtype of our patient which can be grouped under <IIC type a>
according to Shirani J et al classification of "solitary coronary ostium in aorta" is an
exceedingly rare subtype with only single case reported till now. In view of presentation
at this age, prepulmonic course of LAD and improvement with antianginals this
anomaly was thought to be benign and she was advised medical management. Angina
in our patient could be multifactorial like microvascular angina, coronary spasm as
shown in some patients with anomalous LAD or exercise induced ischemia in the
usual LCX territory as reported in patients with super dominant RCA with absent LCX.
This case demonstrates the need for angiography and CTA in establishing a diagnosis
and treatment course. Special care should be taken when evaluating young individuals
with chest pain resembling angina, since sudden death can occur with an anomalous
origin of LCA.
SESSION: GENDER SPECIFIC FEMALE HEART DISEASES
1. A case of Marfans syndrome with ascending and arch of aorta aneurysm
presenting with type A- Dissection of aorta
Dr.E.srikanth ,Dr.Ravi Srinivas MD.DM ,Dr.Adikesava Naidu MD.DM, Dr.y.v.subba
Reddy MD.DM, prof & HOD (cardiology dept. Osmania general hospital ,Hyderabad).
Introduction: Marfans syndrome is a hereditary disease which is Autosomal
dominant inheritance because of mutation in the fibrillin-1 gene, which effects
connective tissue of the body, mainly involves cardiovascular system ,ocular and
skeletal system. It is usually diagnosed with 2010 Revised Ghent Nosology with a
score of more 7.The cardiovascular manifestation include aortic root and arch
aneurysm with high risk of dissection ( root diameter of > 4.5cm) , mitral valve
prolapse , aortic regurgitation secondary to root dilatation, skeletal abnormalities
scoliosis, pectus excavatum, positive thumb & wrist sign, Ectopia lens .
Case report: A female 40yrs old patient presented with chest pain retrosternal since
1month , tearing type radiating neck and back of chest associated with breathlessness
of class 3 on examination conscious and coherent pulse rate = 78/min felt in all limbs
,blood pressure 150/60 mm of Hg CVS S1 S2 ,ESM 3/6 and EDM heard on 3rd left
intercostal area, MDM at the apex. Ecg was showing LV volume overload, on 2Decho
evaluation was showing aorta root showing 4.78cm with severe AR good LV funtion.
patient was evaluated with trans- esophageal echo ,chest x ray and CECT was
diagnosed as ascending and arch aneurysm with dissection of aorta type-A .patient
was referred to CT surgery dept for BENTHAL procedure.
Discussion: The incidence of aortic dissection is estimated to be 2-3.5/10,000
persons per year and peak incidence at sixth and seventh decade with overall mortality
1% /hour ,patients with marfans syndrome at higher risk can occur at younger age.It
is classified into debakey type 1,2,3 and stanfords type A and B depending upon
location of dissection. High clinical suspicion required for diagnosing dissection has
variable clinical manifestation most common is chest pain (80%),severe aortic
insufficiency (45%),hypotension(14%),shock (13%),syncope (12%),MI(7-19%),CVA(8%)
and paraplegia (2%),pulse deficit is seen (26%).The management of the dissection is
beta blocker drug of choice, followed by ACE inhibitors. Medical management is
considered in uncomplicated and chronic type B dissection, surgery is the treatment of
choice acute type A, complicated type B, associated with marfans syndrome, end organ
dysfunction. Endovascular therapy can be done in alternative in complicated type B
dissections.
Conclusion:
Reporting a case of Marfans syndrome with ascending and arch aneurysm with type
A aortic dissection in female of 40yrs old patient.
.
2.
An Unusual Association of Systemic Lupus Erythematosus With Large
Vessel Vasculitis - Dr. Aparna Reddy Sabella
Background: In Systemic lupus erythematosus (SLE) vasculitis is seen in 11 % to 36
% of patients, of which small vessel vasculitis is seen upto 80% and medium vessel
vasculitis in the rest. There are so far only 23 cases of SLE with LVV in literature. We
present a series of 4 such patients which is unique in that aortitis and lupus were
diagnosed at the same time.
Material and methods:
Patients who presented to Rheumatology out-patient department between January
2012 to February 2015 with diagnosis of SLE with LVV fulfilling criteria for SLE were
followed prospectively. Demographic, clinical, laboratory and imageology data was
studied.
Results:
The following table shows the summary of case details of four patients of SLE with
LVV:
All patients were females, between 21-37 years of age. All patients had clinical features
suggestive of SLE with a positive serology for ANA and ds DNA.
CASE DETAILS
Age (Years)
TOTAL DURATION
OF ILLNESS
(MONTHS)
SLEDAI AT
ADMISSION
RENAL DISORDER
CASE 1
21
33
CASE 2
30
4
CASE 3
23
36
CASE 4
37
60
31
16
17
24
PRESENT
-
PRESENT
NEUROLOGIC
DISORDER
CLAUDICATION
OF EXTREMITIES
BRUIT OVER
SUBCLAVIAN
ARTERIES OR
AORTA
ESR in mm Hg at
1st Hour
ANTICARDIOLIPIN
ANTIBODIES
COMPLEMENTS
BIOPSY FINDINGS
-
Class V lupus
nephritis
-
PRESENT
-
YES
YES
YES
YES
YES
YES
YES
YES
3
68
80,106
70
negative
negative
Acl IgG -42 (positive)
Not done
low
-
Not done
-
CT angiogram i st
and 2nd part of
subclavian artey
entire left radial artey
MRA, irregularity of
supraclinoid segment
Doppler
showed
reduced
flow b/l
upper
limb
IMAGING
FINDINGS
low
-
low
Renal-class
Vnephritis
CT angio –
CT angiogram
circumfurantial segment
ly thickened
stenosis
desending
(7cm) of
aorta left
descending
subclavian
thoracic
artery
aorta, upto
level of aortic
hiatus with
post stenotic
dilatation,
stenosis of
second part of
left
subclavian
artey with
irregularity of
of the wall.
of right ICA,
cavernous segment of
left ICA, cortical
branches of left MCA
& left A1
ACA,Multiple focal
narrowings with
contour irregularity
noted in bilateral
vertebral
arteries,basilar rtery
& PCAs
arteries,
bilateral
brachial,
radial,
femoral,
dorsalis
pedis,
posterior
tibial.A.
DOSE OF
STEROIDS
Pulse methyl
prednisolone (1
gm for 3 days),
followed by
1mg/kg
1mg/kg oral steroid
1mg/kg
oral
steroid
IMMUNOSUPPRES
SION
Inj
Cyclophospha
mide CD 4500mg,
followed by
Azathioprine
75 mg
Pulse methyl
prednisolone
(1 gm for 3
days),
followed by
1mg/kg
Inj
Cyclophospha
mide CD 4500mg,
followed by
Azathioprine
50 mg
Inj
Cyclophosphamide
CD -3600mg,
followed by
Azathioprine 100 mg
Previously
tab
Methotrex
ate 15 mg
once a
week
-
-
-
INTERVENTION
PTCA to
descending
aortas in Jan
2013,
Now on
Tab
Azathiopri
ne 50 mg
PTA to left
subclavian
artery in Dec
2014
SLEDAI* at follow
up
8
0
0
0
OUTCOME
Disease relapse
and
reintervention
Disease in
remission
Disease in remission
Disease is
active
TOTAL DURATION
OF FOLLOW UP
(MONTHS)
30
14
12
60
*Systemic Lupus Erythematosus Disease Activity Index
Conclusion:
Though rare, we found an association of SLE with LVV emphasizing the need for high
clinical suspicion and early aggressive therapy.
2. Ovarian Cyst Bleed Due To Anticoagulants - Dr Suma Latha
Introduction
Haemorrhage into ovarian cysts is a frequent and potentially life threatening
complication in women on anti coagulation therapy. We report a case of 19 year old
girl, a case of CRHD with mitral valve replacement done 1 year back on anti
coagulation therapy , with SOB, pain abdomen, mild abdominal distension and
hypotension. USG showed acute right haemorrhagic cyst.
A 19 year old unmarried girl reported to emergency room on 13/12/14 at 10 pm with
pain abdomen since 3 days, SOB since 1 day. There was history of fever with vomiting
on & off. She is a K/C/O CRHD with severe MR, Mitral valve prolapse diagnosed at 7
yrs age on treatment, underwent mitral valve replacement in 2013 at Gandhi hospital.
From then she is on treatment with tab. Acitrom 2mg/4mg on alternate days &
penicillin prophylaxis.
O/E , pt c/c, Pallor +++, PR-110/mn, BP-90/60mm of Hg, H/L-S1 S2+,PSM+, P/Amild distension ,tenderness in lower abdomen. USG-Acute right hemorrhagic cyst6.7x5.2cm. Gross ascites. Paracentesis- blood stained fluid aspirated.
Date13/12
Hb
WBC
platelets
PT
INR
9.00AM
4.6
4700
1.58
LAKHS
60
SEC
5.15
APTT
10.30 PM
3.3
6200
1.4
LAKHS
>70
SEC
>10
84
SEC
Treatment given- Antibiotics,vit-K,5 FFP’s, 4 packed cells transfused.
PT
25.4
INR
2.24
APTT
38.5
2 FFP’s transfused again.
Emergency laparotomy done- 3.5 litres of haemoperitoneum+,500gms clots+,Rt
hemorrhagic cyst of 6x7 cm involving rt ovary- rt oophorectomy done. Both tubes &left
ovary normal. Intraoperatively - 1 packed cell given. Post op-2 packed cells, 4 FFP’s
given. Anti coagulants started after. Post op –uneventful.
Discussion
Risk for major bleeding with coumarin
Coumarin (a vitamin K antagonist) is commonly used in patients with ischemic stroke,
prosthetic heart valves, atrial fibrillation, ischemic heart disease, and VTE. The wide
ranges of risk associated with vitamin K antagonists occur because the bleeding risk
depends on several factors, including intensity of anticoagulant effect, patient
characteristics (particularly age), the concomitant use of drugs that interfere with
hemostasis, and the length of therapy.
Blood product transfusion
Transfusion of blood products, usually fresh frozen plasma (FFP), is the most
commonly used method for rapidly reversing coumarin. The usual dose of FFP for
anticoagulant reversal is 15 mL/kg of body weight. Irrespective of the blood product
administered to reverse coumarin anticoagulation, the patient should also receive
intravenous vitamin K at a dose of 2.5 to 5 mg administered over 30 minutes;
otherwise, the INR is unlikely to completely correct and a “rebound coagulopathy” may
develop after the transfused factors are
cleared.
How to prevent such a catastrophy? - proper counselling of the patient regarding
proper drug intake, interactions, side effects, need of regular follow up with PT,INR.
Conclusions
Anticoagulant therapy is one of the most widely used medical therapies. The classic
anticoagulants, UFH and coumarin, have reasonable efficacy and safety, and specific
antidotes, but have certain undesirable features. . Ironically, newer anticoagulants
developed to overcome these limitations do not have specific antidotes, requiring
careful patient selection and dosing to optimize their therapeutic benefits. This group
includes LMWHs, specific factor Xa inhibitors, and DTIs. . Additional research on
reversal agents or techniques for the newer anticoagulants is needed, including
adequately powered clinical studies. . In the setting of major bleeding, cessation of
anticoagulation therapy and, if possible, reversal of anticoagulation effects, using
available, specific reversal agents. In some cases, the use of specific blood products
may be necessary.
SESSION : OTHERS
1. Case of young Hypertension - Dr. Sunita Dinakar Kumbhalkar
15 year old female, c/o Weakness of both lower limbs – 2 months. O/E - Systemic HT,
Pallor, Lt cervical and abdominal paraumbilical midline masses, Paraparesis with
contractures in both lower limbs
Investigations
Hb - 5 gm %, severe microcytic, hypochromic anaemia. biochemical investigations –
normal. X ray chest -superior mediastinal widening. CT (thorax and abdomen)multiple mediastinal and retroperitoneal masses encasing aortic arch, abdominal
aorta, IVC and its branches, a 3.8 ×3.4×2.8 cm cervical mass, 4.4×3.4 cm mediastinal
mass was noted displacing SVC superoanteriorly. Lytic, sclerotic lesions suggestive of
metastasis were noted in dorsolumber vertebrae, sternum, sacrum, iliac bones and
both femoral heads. Remaining intraabdominal organs were normal.
Histopathology of cevical mass - metastasis from paraganglioma
Immunohistology - positive for Synaptophysin and chromogranin
Discussion
5% to 10%- extra-adrenal sites, upper cervical region to the pelvis, parallel to the
autonomic nervous system. Malignant PGL defined by the presence of metastasis are
very rare and aggressive tumours. Head and neck PGLs are usually clinically silent
whereas abdominal sPGLs are usually catecholamine secreting. Abdominal
paragangliomas – retroperitoneal, 85% of all extra adrenal paragangliomas.20%
malignant . The most common site for retroperitoneal PGL- between the origin of
inferior mesenteric artery and the aortic bifurcation known as organ of Zuckerkandl.
Paragangliomas – jugulotympanic body - chemodectomas, paragangliomas carotid
body- carotid body tumours. Paragangliomas located in the second part of duodenum
are called gangliocytic paraganglioma.
C/F depends on the variability of these tumours which can express different
catecholamines, biosynthetic enzymes, secrete different vasoactive peptides( ANP,
neuropeptide Y, adrenomodullin) . symptoms related to tumour mass or present
different symptoms related to other organ involvement in syndromic forms.
Hypertension is the most common feature of secretary PGL. Systemic symptoms like
anorexia, fatigue, weight loss and clinical features related to metastatic disease such
as pain in bones are common
Diagnostic modalities
Biochemical- urinary and plasma metanephrines –99% sensitivity. Radiological- CT ,
MRI, Radioisotope study Histopathological – confirmatory with
immunohistochemistry , Genetic studies
Treatment
Surgery with complete removal of mass either via laparoscopy or via traditional
laparotomy is the treatment of choice for retroperitoneal paragangliomas owning to its
malignant potential. Patients with metastatic disease will require adjuvant
radiotherapy while chemotherapy is restricted to patients not accessible for surgery
and resistant to radionuclide therapy
Take Home Message
•
•
•
•
Rare presentation
High index of suspicion
Importance of measuring blood pressure in all age groups
Multidisciplinary approach (physician, radiologist, pathologist, anaesthetist and
surgeon )
2. Mitral Valve Repair for Mitral Regurgitation in a patient with Systemic Lupus
Erythematosus-A Case Report – Dr. Palanki Surya Satya Gopal
Introduction:
Mitral valve regurgitation due to Systemic Lupus Erythematosus (SLE) is a rare cause
of valvular heart disease, necessitating valve surgery. Currently, there are only 47 case
reports in the world medical literature of mitral valve replacement or repair in patients
who have lupus. Immunologic insult plays a fundamental role in its pathogenesis.
Case report:
A 13 year old girl presented with exertional breathlessness and pedal oedema since 6
months. Patient was a known case of SLE for which she was being treated with oral
steroids since 18 months. Patient had no history of Rheumatic fever in the past. Her
physical exam revealed a
blowing systolic murmur radiating to the left axilla. On
evaluation found to have Severe Mitral Regurgitation(MRJA:12cm2), Mild RV
dysfunction and Normal LV function.ANA and anti ds-DNA were positive. Patient was
taken up for elective surgery after initial medical stabilisation and control of SLE
disease activity. Mitral valve repair done with Teflon felt as leaflets were pliable and
sub valvular apparatus was spared . Post operatively patient had acute kidney injury
was managed with peritoneal dialysis and steroids. Post operative Echocardiography
revealed mild to moderate MR (6cm2). Patient was discharged on fifteenth post
operative day.
Conclusion:
The experience with mitral valve surgery in SLE patients is limited. If there is localized
abnormality in structure and function of mitral valve in relatively stable and controlled
SLE patient, valve repair is a better choice. Although timely diagnosis is essential to
prevent progression of valvular lesions, treatment remains a challenge because of the
lack of large systematic studies.
3. Radiotherapy in patients with implanted Cardiac Pacemaker – Dr.Deepti
Baliyaveettil.
Introduction
Breast cancer is the most frequently diagnosed life-threatening cancer in women and
the leading cause of cancer death among women worldwide. Primary treatment for
breast cancer is surgery and radiation therapy, along with adjuvant hormone or
chemotherapy when indicated. Radiation therapy may follow surgery in an effort to
eradicate residual disease while reducing recurrence rates. With the increase in
prevalence of cardiac morbidity, patients with pacemakers requiring radiotherapy has
increased. Pacemakers are usually implanted subcutaneously, overlying the pectoral
muscles and thus may be affected by radiation used in treatment of cancers of breast,
lung, chest wall and lymphomas. Therapeutic irradiation may cause pacemaker
malfunction due to the effect of ionizing radiation or electromagnetic interference. Most
cited guidelines for the management of radiation oncology patients are American
Accosiation of Physicists in Medicine (AAPM) guidelines. They recommend that these
patients should not be treated with a betatron, pacemakers should not be in the direct
treatment field, to limit the accumulated dose to the pacemaker to 2 Gy. The patient’s
coronary and pacemaker status should be assessed by cardiologist before and soon
after the completion of radiotherapy. Patient should be monitored before and after
treatment. Pacemaker function should also be assessed regularly
Case Summary
We present two cases with carcinoma breast with implanted pacemaker who received
adjuvant radiotherapy at NIMS.
Case I : 41yr old female, Permanent pacemaker placement in Dec 2012. Diagnosed as
Ca Right breast and Underwent Right MRM in Apr 2013 and received adjuvant
chemotherapy. Planned for Adjuvant Radiotherapy to Rt Chest wall and Right SCF. No
local recurrence or distant metastasis. Cardiology consultation taken , detailed 2D
Echo done, reprogramming done before starting RT.
Treatment Plan : Adjuvant RT by 3DCRT to right chest wall and right SCF, 2Gy/fr,25
fractions to a total dose of 50 Gy. Dose to pace maker was restricted to 0.6Gy mean
and max of 1.8Gy. Patient was monitored daily with ECG , cardiology review. Doing
well on follow up
Case II: 68 yr old female, non diabetic, non hypertensive. History of pacemaker
implantation 7 yrs ago for sick sinus syndrome. Patient on 6 monthly pacemaker
programming. She was diagnosed with Carcinoma left breast in January 2014 and
underwent left modified radical mastectomy on 9th January 2014. She received
chemotherapy and was referred for adjuvant radiotherapy to radiation oncology. Pre
treatment ECG showed incomplete Right bundle branch block and 2DEcho showed
normal study. She had good performance status and had no evidence of loco-regional
recurrence clinically or metastasis. Cardiology consultation taken , detailed 2D Echo
done, reprogramming done before starting RT. After explaining the consequences to the
patients and attendant and with their consent the treatment was given.
Treatment Plan: Adjuvant RT by 3DCRT to left chest wall and left SCF, 2Gy/fr,25
fractions to a total dose of 50 Gy. Dose to pace maker was restricted to 0.7Gy mean
and max of 2.2Gy. Patient was monitored with ECG, pacemaker function assessment
regularly. Patient doing well on follow up
Conclusions
Planning radiotherapy to minimize the dose, close monitoring throughout the
treatment and with strong support from the Cardiologists these patients can receive
adequate treatment.