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Table of Contents
Year : 2019  |  Volume : 9  |  Issue : 3  |  Page : 171-173

A rare association of congenital mid-muscular ventricular septal defect with acute anterior wall myocardial infarction in the elderly: Clinical and hemodynamic implications

1 Department of Cardiology, Yashoda Hospitals, Raj Bhavan Road, Somajiguda, Hyderabad, Telangana, India
2 Ekbote's Clinic, Kachiguda Station Road, Chappal Bazar, Hyderabad, Telangana, India

Date of Web Publication3-Dec-2019

Correspondence Address:
Dr. Pankaj Jariwala
Department of Cardiology, Yashoda Hospitals, Raj Bhavan Road, Somajiduda, Hyderanad - 500 082, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_23_19

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We report a rare association of acute myocardial infarction (AMI) in an elderly patient who had undiagnosed mid-muscular ventricular septal defect (VSD). The patient underwent primary percutaneous coronary angioplasty of the left anterior descending artery. It is uncommon to survive with VSD at this age, and the association of it with AMI is still rare. Whether the presence of congenital heart disease poses them for the increased risk of coronary artery disease is a matter of further investigation. Furthermore, the presence of VSD while they incur myocardial infarction affects the prognosis of the patient is not clear.

Keywords: Acute myocardial infarction, muscular ventricular septal defect, primary percutaneous coronary angioplasty, ventricular septal rupture

How to cite this article:
Jariwala P, Ekbote A. A rare association of congenital mid-muscular ventricular septal defect with acute anterior wall myocardial infarction in the elderly: Clinical and hemodynamic implications. J Indian coll cardiol 2019;9:171-3

How to cite this URL:
Jariwala P, Ekbote A. A rare association of congenital mid-muscular ventricular septal defect with acute anterior wall myocardial infarction in the elderly: Clinical and hemodynamic implications. J Indian coll cardiol [serial online] 2019 [cited 2022 Sep 24];9:171-3. Available from: https://www.joicc.org/text.asp?2019/9/3/171/272168

  Introduction Top

The presence of congenital heart diseases is rare in the elderly population, although there are case reports of their occurrence. Congenital heart diseases can reduce the long-term survival but their milder forms can give them better survival chances. The development of coronary artery disease was studied as some of them may lead to exaggeration of risk factors such as hypertension in coarctation of the aorta.

We report such an association of acute myocardial infarction (AMI) and congenital mid-muscular ventricular septal defect (VSD). The survival with mid-muscular VSD in the elderly is rare through the natural history of VSD. The development of coronary artery disease in the presence of congenital heart disease whether it is an additional risk factor is not known. The presence of VSD that alters hemodynamics in the post-myocardial infarction period may act as a “release valve” in diastole. Whether the prognosis of the patient with AMI in the presence of VSD has any survival advantage also needs to be ascertained.

Although a rare association, it poses many questions related to their significance in terms of prognosis and hemodynamic implications.

  Case Report Top

A 72-year-old male hypertensive admitted with complaints of retrosternal chest discomfort for the last 2 days. His vital parameters recorded were a pulse rate of 112/min and a blood pressure of 80/60 mmHg. Electrocardiogram showed ST-segment elevation in leads V1–V6, with reciprocal ST-segment depression in leads II, III, and aVF. Echocardiography showed regional wall motion abnormality of the distal interventricular septum and anterior wall. There was small mid-muscular VSD (4–5 mm) with the pulmonary to systemic cardiac output being found to be Qp/Qs =1.4/1 [Figure 1]a. The left ventricular to right ventricular (LV to RV) gradient across the VSD was 17.6 mmHg [Figure 1]b. The RV was normal with moderate pulmonary arterial hypertension (RV systolic pressure =60 mmHg). In addition, there was mild concentric LV hypertrophy with mild left atrial dilatation. The mid-muscular VSD location was unusual for the occurrence of acute ventricular septal rupture (VSR). Hence, we decided for coronary angiography after adequate dual-antiplatelet agent and high-intensity statin. The patient had ongoing chest pain while angiography, which showed complete occlusion of the left anterior descending (LAD) artery [Figure 2]a. His dominant left circumflex artery had nonobstructive lesions, and the mid-segment of nondominant right coronary artery had 90% stenosis [Figure 2]b. We did percutaneous coronary intervention, and two sirolimus-eluting stents were deployed after predilatation [Figure 2]c and d], which restored TIMI III flow in the LAD, and the patient had complete relief of his chest pain. His blood pressure improved to 110/70 mmHg. The patient was discharged on optimal medical management, which includes angiotensin-converting enzyme inhibitor, beta-blocker, eplerenone, dual-antiplatelet agents, and high-intensity statin and has completed an 8-month follow-up without any symptoms.
Figure 1: Two-dimensional echocardiography using color flow mapping showing moderate restrictive mid-muscular ventricular septal defect (solid arrow, a) with left to right shunt. The continuous-wave Doppler (inverted) across ventricular septal defect revealed gradient of 17.6 mmHg suggestive of left to right shunt (b)

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Figure 2: Coronary angiography showing thrombotic occlusion of the proximal segment of the left anterior descending artery (solid arrow) with TIMI 0 flow and the dominant left circumflex artery had nonobstructive lesions (right anterior oblique caudal view, a). The nondominant right coronary artery had 90% stenosis of mid-segment (b). Primary angioplasty was done, and two drug-eluting stents (dashed arrows) were deployed which restored TIMI 3 flow in right anterior oblique caudal and left anterior oblique caudal views (c and d)

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  Discussion Top

Clinically, the mid-muscular VSD and AMI is a rare association. Initially, we were confused about the nature of VSD, whether it was post-myocardial infarction VSR or congenital mid-muscular VSD. However, after discussion among our “heart team,” we thought that it was congenital VSD as it had circumscribed edges, located in the mid-segment of the interventricular septum, while acquired VSR is formed near apical segments of the LV in most of the cases of acute anterior wall infarction. The presence of VSR carries high mortality rate, and its management strategy differs. Although surgical repair with revascularization is the preferred strategy for VSR, interventional management can be considered in the form of device closure of VSR.

The prevalence of congenital VSD in the adults is reported in 10% of cases;[1] after the age of 70 years, it is very rare and there are few cases of congenital VSDs in the elderly.[2] Mostly, these VSDs are smaller and have shunt <1.5:1 and hence asymptomatic till the seventh decade of life. Eighty-five percent of patients survive until the second decade with significant shunt; however, after 60 years, only 25% of patients survive.[3] Small VSDs, as in our case, are not always benign and may develop arrhythmias, infective endocarditis, and sudden cardiac death.[2]

The development of the valvular heart disease or coronary artery disease in the patients of stable adult congenital heart disease leads to hemodynamic derangements, which change asymptomatic state to symptomatic clinical presentations ranging from angina to heart failure. Congenital heart diseases in adults do not directly predispose them for the development of coronary artery disease, except in coarctation of the aorta secondary to persistently elevated blood pressure.[4],[5] VSD, as in our case, does not lead to increase the risk of coronary artery disease and the development of associated CAD has the same risk as in the general population. Fyfe et al. studied coronary atherosclerosis in the patients of cyanotic and acyanotic congenital heart disease patients who were operated and remained without any corrective surgery. They did coronary angiography and necropsy studies in few patients and found that there are anti-atherogenic factors such as hypoxemia, low platelet count, and hyperbilirubinemia as coronaries were dilated and free of atheromas.[6]

On the contrary, Lui et al. studied the risk of developing atherosclerotic cardiovascular disease (ASCVD) in patients with adult congenital heart diseases.[7] The 10-year risk of ASCVD is more in this population as the age advances secondary to the risk factors such as obesity, hypertension, hyperlipidemia, diabetes mellitus, and elevated Hs-CRP are more common in them.[8] Hence, they need regular screening and management of risk factors to prevent the development of ASCVD.

The AMI in the presence of VSD was described in an elderly patient who underwent coronary artery bypass grafting and the correction of VSD.[9] Sieweke et al. reported the association of double-outlet LV with subpulmonary stenosis and AMI in a 70-year-old elderly patient.[10]

Whether the presence of VSD in the patient with AMI confers any survival advantage is not clear.

  Conclusion Top

It is rare for the development of AMI in an elderly patient who has survived through the natural history of congenital mid-muscular VSD. This association is not reported in the literature. It carries some message to clinicians related to hemodynamic implications and their long-term prognosis, which is not studied in detail. We need further research in this area at least some registry data, which will enlighten us further in this gray area.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mahoney LT. Acyanotic congenital heart disease. Atrial and ventricular septal defects, atrioventricular canal, patent ductus arteriosus, pulmonic stenosis. Cardiol Clin 1993;11:603-16.  Back to cited text no. 1
Patrianakos AP, Parthenakis FI, Chrysostomakis SI, Vardas PE. Ventricular special defect in the elderly: An uncommon clinical entity. Hellenic J Cardiol 2005;46:158-60.  Back to cited text no. 2
Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O'Fallon WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation 1993;87:I38-51.  Back to cited text no. 3
Yalonetsky S, Horlick EM, Osten MD, Benson LN, Oechslin EN, Silversides CK, et al. Clinical characteristics of coronary artery disease in adults with congenital heart defects. Int J Cardiol 2013;164:217-20.  Back to cited text no. 4
Giannakoulas G, Dimopoulos K, Engel R, Goktekin O, Kucukdurmaz Z, Vatankulu MA, et al. Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors. Am J Cardiol 2009;103:1445-50.  Back to cited text no. 5
Fyfe A, Perloff JK, Niwa K, Child JS, Miner PD. Cyanotic congenital heart disease and coronary artery atherogenesis. Am J Cardiol 2005;96:283-90.  Back to cited text no. 6
Lui GK, Rogers IS, Ding VY, Hedlin HK, MacMillen K, Maron DJ, et al. Risk estimates for atherosclerotic cardiovascular disease in adults with congenital heart disease. Am J Cardiol 2017;119:112-8.  Back to cited text no. 7
Roche SL, Silversides CK. Hypertension, obesity, and coronary artery disease in the survivors of congenital heart disease. Can J Cardiol 2013;29:841-8.  Back to cited text no. 8
Tayama E, Fujita S, Ueda T, Imasaka KI, Enomoto N, Onitsuka H, et al. Ventricular septal defect in an octogenarian: A case report of VSD surgical repair concomitant with coronary artery bypass and valvular surgery. Case Rep Cardiol 2012;2012:429569.  Back to cited text no. 9
Sieweke JT, Westhoff-Bleck M, Napp LC, Avsar M, Vogel-Claussen J, Schäfer A, et al. Myocardial infarction in grown up patients with congenital heart disease: An emerging high-risk combination. Int J Cardiol 2016;203:138-40.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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