Case Reports Delayed Presentation of Ventricular Septal Rupture – A Case Report M Ullah, L A Sayami, SK Chakrabarty, AAS Majumder Department of Cardiology, NICVD, Dhaka Abstract: Keywords: Ventricular septal rupture, Myocardial infarction. Ventricular septal rupture (VSR) is a devastating complication of acute MI. In GUSTO trial about 0.2% patient developed VSR.1 The mortality rate among patients with septal rupture who are treated conservatively without mechanical closure is approximately 24 percent in the first 24 hours, 46 percent at one week, and 67 to 82 percent at two months. We are reporting a case of VSR with MI who presented with heart failure after 14 months of index MI. Case Report: Mr. X a 75 years old farmer presented with shortness of breath and swelling of both lower limbs for two months. According to his statement he was reasonably well about two months back. Then he developed severe respiratory distress which is of NYHA II grade with edema of both lower limbs. It was not associated with any chest pain. His shortness of breath increased in last two days. He was normotensive, nondiabetic and exsmoker. He gave history of sudden severe chest pain about 14 months back. At that time he took some medication (name of the drugs and other documents not available) and continued for few days. Chest pain subsided after the initial 24 hours. After that he continued his usual activities without any limitation. (Cardiovasc. j. 2009; 2(1) : 91-94) sided pleural effusion. Echocardiogram revealed aneurysm in the posterior part of the basal portion of the IVS with rupture in the RV. It was a type III variety of ventricular septal rupture. Cardiac catheterization showed significant step up of oxygen saturation in the RV with QP/QS ratio of 2: 1. Coronary angiogram revealed 100% stenosis of the RCA. Distal RCA and PDA are visualized by collaterals from LAD. LAD and LCX were normal. Patient was treated with diuretics, vasodilator, antiplatelet and antilipid drugs and was referred for surgical closure of VSR with CABG. On examination, his blood pressure was normal, but he had tachycardia, dependant oedema and raised JVP. His apex beat was shifted. There was pansystolic murmur of Grade 3/6 in left lower parasternal area. He had hepatomegaly and bilateral basal crepitations in lungs. There was no feature suggestive of deep vein thrombosis (DVT) of lower limbs. ECG shows Q wave in II,III & aVF without any change in ST – segment. Cardiac markers were normal. X-ray showed cardiomegaly with right Fig.-1: Aneurysmal dilatation of IVS. Address of Correspondence : Dr. Mohammad Ullah, Assistant Professor, Dept. of Cardiology, National Institute of Cardiovascular Diseases, Dhaka, Bangladesh. Cardiovascular Journal Volume 2, No. 1, 2009 Discussion: Acute myocardial infarction (AMI) may be associated with devastating mechanical complications. Rupture of the interventricular septum (VSR) is one of them. In the era before reperfusion therapy, septal rupture complicated 1 to 3 percent of AMI.2-6 In GUSTO -1 trial (41,021 patients with thrombolytic therapy) VSR was suspected in 0.3% cases and confirmed in 0.2% cases.1 thus reperfusion therapy has reduced the incidence of VSR. VSR was first reported by Latham in 1846. 7 Without reperfusion therapy VSR usually occurs in the first week. 3,5, 8,9,10 The median time from the onset of symptoms of AMI to rupture is generally 24 hours or less in patients who are receiving thrombolysis.22 Fig.-2: Aneurysmal dilatation of IVS. VSR occurs more frequently in anterior than other types of AMI.2, 6, 8,11,12. In our patient it was an inferior MI. Risk factors for VSR in the era before thrombolytic therapy included HTN,13,14 advanced age (60 to 69 years),11 female sex,13,15 and the absence pf history of angina or MI.1, 2, 16–18 Angina or infarction may lead to myocardial preconditioning as well as to the development of coronary collaterals, both of which reduce the likelihood of septal rupture. 18 In patients undergoing thrombolysis, advanced age, female sex and the absence of smoking are often associated with an increased incidence of septal rupture,6 whereas the absence of antecedent angina has not been associated with an increased risk.11 Our patient was an elderly patient of 75 yrs age. Fig.-3: Color flow from aneurysm to RV. Becker and van Mantgem classified the morphology of free-wall rupture into three types, which are also relevant to ventricular septal rupture19 : in type I there is an ruptures have an abrupt tear in the wall without thinning; in type II, the infarcted myocardium erodes before rupture occurs and is covered by a thrombus; and type III has marked thinning of the myocardium, secondary formation of an aneurysm, and perforation in the central portion of the aneurysm. In our patient it was a type III VSR. The size of septal rupture ranges from millimeters to several centimeters. Morphologically, septal rupture is categorized as simple or complex. Simple septal rupture has a discrete defect and a Fig.-4: Color flow from aneurysm to RV. 92 Delayed Presentation of Ventricular Septal Rupture – A Case Report direct through-and-through communication across the septum. The perforation is at the same level on both sides of the septum. Extensive hemorrhage with irregular, serpiginous tracts within necrotic tissue characterizes complex septal rupture. 7,16 Septal ruptures in patients with anterior myocardial infarction are generally apical and simple . Conversely, in patients with inferior myocardial infarction, septal ruptures involve the basal inferoposterior septum and are often complex. In our patient the VSR was in inferoposterior and basal portion of IVS. Ventricular septal ruptures associated with an inferior or anterior myocardial infarction generally involve right ventricular infarction.15 M Ullah et al. reported a mortality rate of only 6 percent among patients who survived the first 30 days after surgery.7 Among 60 patients who survived surgical repair, the 5-year survival rate was 69 percent, the 10-year survival rate was 50 percent, and the 14-year survival rate was 37 percent.22 Eighty-two percent of these patients were in New York Heart Association class I or II at follow-up, and angina and other medical problems were not prevalent. The development of a residual or recurrent septal defect is reported in up to 28 percent of patients who survive repair and is associated with high mortality.21 References: Some studies have found that septal rupture is associated with multivessel coronary artery disease.2, 8 However, others found a high prevalence (54 percent) of single-vessel disease among patients with ventricular septal rupture.11,20 Ventricular septal rupture is likely to be associated with total occlusion of the infarct- related artery.3,6,11 In our patient it was total occlusion of the RCA and LAD & LCX were normal. In the GUSTO-I study, total occlusion of the infarct-related artery was documented in 57 percent of patients with ventricular septal rupture, as compared with 18 percent of those without ventricular septal rupture.6 Collaterals are less often evident in patients with ventricular septal rupture,2, 11, 18 supporting the hypothesis that collateral circulation reduces the risk of rupture of the cardiac free wall as well as septal rupture.18 The mortality rate among patients with septal rupture who are treated conservatively without mechanical closure is approximately 24 percent in the first 24 hours, 46 percent at one week, and 67 to 82 percent at two months. 3,4 Lemery et al. reported a 30-day survival rate of 24 percent among medically treated patients, as compared with a rate of 47 percent among those treated surgically.20 Our patient survived 14 months after the MI having some medication only during the initial few months. To the best of our knowledge, this is the first reported case of VSR in our country who survived for such a long period with conservative treatment For patients who survive surgery, the long-term prognosis is relatively good.7, 21Crenshaw et al. 93 1. Crenshaw BS, Granger CB, Birnbaum Y, et al. Risk factors, angiographic patterns, and outcomes in patients with ventricular septal defect complicating acute myocardial infarction. Circulation 2000; 101: 27-32. 2. Pohjola-Sintonen S, Muller JE, Stone PH, et al. Ventricular septal and free wall rupture complicating acute myocardial infarction: experience in the Multicenter Investigation of Limitation of Infarct Size. Am Heart J 1989; 117: 809-18. 3. Radford MJ, Johnson RA, Daggett WM Jr, et al. Ventricular septal rupture: a review of clinical and physiologic features and an analysis of survival. Circulation 1981; 64: 545-53. 4. Topaz O, Taylor AL. 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