|Year : 2020 | Volume
| Issue : 2 | Page : 148-150
Recurrent palpitation and supraventricular tachycardia in a patient with a restrictive ventricular septal defect
Abubakar Yerima1, Bello Abdullahi Ibrahim2, Faruk Buba1, Mohammed Abdullahi Talle1
1 Department of Medicine, College of Medical Sciences, University of Maiduguri, Maiduguri, Nigeria
2 Department of Paediatrics, College of Medical Sciences, University of Maiduguri, Maiduguri, Nigeria
|Date of Submission||09-Jun-2020|
|Date of Decision||15-Jul-2020|
|Date of Acceptance||27-Jul-2020|
|Date of Web Publication||13-Nov-2021|
Dr. Abubakar Yerima
Department of Medicine, College of Medical Sciences, University of Maiduguri, Maiduguri
Source of Support: None, Conflict of Interest: None
Ventricular septal defect (VSD) is the most commonly recognized congenital heart disease. The presentation in patients with VSD is variable depending on the size, location, hemodynamic consequences, and complications including bacterial endocarditis, pulmonary hypertension, and cardiac arrhythmias. The most common cardiac arrhythmias in unoperated VSD are premature ventricular contractions (isolated, couplets, and multiform). We present a case of supraventricular tachycardia, an uncommon occurrence, presenting with recurrent syncope in a 42-year-old female with restrictive VSD.
Keywords: Palpitation, restrictive ventricular septal defect, supraventricular tachycardia
|How to cite this article:|
Yerima A, Ibrahim BA, Buba F, Talle MA. Recurrent palpitation and supraventricular tachycardia in a patient with a restrictive ventricular septal defect. Nig J Cardiol 2020;17:148-50
|How to cite this URL:|
Yerima A, Ibrahim BA, Buba F, Talle MA. Recurrent palpitation and supraventricular tachycardia in a patient with a restrictive ventricular septal defect. Nig J Cardiol [serial online] 2020 [cited 2021 Dec 5];17:148-50. Available from: https://www.nigjcardiol.org/text.asp?2020/17/2/148/330419
| Introduction|| |
Ventricular septal defects (VSDs) account for 20% of all congenital cardiovascular disorders and 10% of those diagnosed in adults., The estimated prevalence is 0.5 per 1000 of the adult population and is increasing because of improved detection rate. It constituted 26.1% of all congenital heart diseases diagnosed in our center.
The hemodynamic and physiologic sequalae of VSD are determined by size of the defect and the relative resistance in the pulmonary and systemic vasculature. In restrictive VSD (small defects), there is little or no functional alterations contrary to larger defects in which there is an initial left-to-right shunting that declines with a progressive increase in pulmonary vascular resistance, culminating in right-to-left shunting when pulmonary vascular resistance becomes supra-systemic.
The presentation in patients with VSD depends on the type of defect, size, associated cardiovascular abnormalities, and pulmonary vascular resistance. Patients with small defects and normal pulmonary artery systolic pressures remain asymptomatic, while those with large defects develop left ventricular failure, pulmonary hypertension, and right ventricular failure. The prognosis in adults with small VSD and normal pulmonary pressure is excellent, and only endocarditis prophylaxis is required. Surgical closure is indicated in these categories of patients after the second episode of endocarditis. The decision to close medium-to-large VSD in adults depends on numerous factors including findings at cardiac catheterization.
The incidence of cardiac arrhythmia is high in patients with VSD. Ventricular arrhythmias are more common, mostly in the form of premature ventricular contractions, couplets, or multiform. Ventricular tachycardia and sudden cardiac death were reported in 5.7% and 4.0%, respectively., Atrial fibrillation is the most common supraventricular arrhythmia and is more common in patients with hemodynamically significant VSD resulting in structural cardiac alterations, especially atrial enlargement and following repair. Atrioventricular node-dependent supraventricular tachycardia (SVT) is uncommon in patients with isolated VSD. We present a case of recurrent palpitation and SVT in a 42-year-old female patient with restrictive peri-membranous and muscular VSDs who remained symptomatic after repair.
| Case Report|| |
A 42-year-old lady presented to the emergency room (ER) on the April 23, 2015, with sudden onset of palpitation, shortness of breath, and central chest discomfort. She gave a history of lightheadedness, perspiration, and a feeling of impending doom but no syncope. She has had self-limiting paroxysms of palpitation and shortness of breath since adolescence, but episodes are becoming more frequent and distressing in the recent months. She is not hypertensive or diabetic and does not smoke cigarettes or drink alcohol. She is para 6 and had her last child about 3 years prior to presentation.
When examined, she was anxious and profusely sweaty with cold extremities. The pulse rate was 160 beats per minute (bpm) in the emergency room (ER), but blood pressure was not measurable using a mercury sphygmomanometer. Jugular venous pressure was not elevated and point of maximum impulse was in the fifth left intercostal space. She had a 4/6 holosystolic murmur maximum in the third intercostal space left of the sternal border. There were no features of pulmonary hypertension or aortic regurgitation. A clinical diagnosis of symptomatic VSD was made.
A 12-lead resting electrocardiogram (ECG) obtained 30 min later showed a sinus rhythm at 83 bpm with depressed ST-segments and T-waves. There was no chamber enlargement, QT prolongation, or epsilon wave. Transthoracic echocardiography showed peri-membranous VSD measuring 6 mm and partially occluded by tricuspid tissue and muscular VSD measuring 5 mm on the left ventricular side, about 19 mm from the membranous VSD. The membranous VSD had a peak systolic velocity of 4.5 m/s and pressure gradient of 75 mmHg. Cardiac chambers and functions were normal. There was no pulmonary hypertension or aortic regurgitation. Other cardiac valves were normal, and no other congenital defect was detected. She was referred to India where a patch closure of the membranous VSD was done on October 10, 2015, leaving the muscular VSD unrepaired, and was discharged on metoprolol 25 mg BD and aspirin 81 mg OD.
She presented to the ER on November 7, 2015, with paroxysmal palpitation, shortness of breath, and chest pain a month after the VSD repair. ECG revealed regular narrow complex tachycardia of 233 bpm [Figure 1], consistent with SVT. She spontaneously reverted to sinus rhythm while in the intensive care unit.
|Figure 1: Electrocardiogram tracing of the patient with supraventricular tachycardia and ventricular septal defect|
Click here to view
| Discussion|| |
Despite an improvement in various corrective operations, most patients in sub-Saharan Africa go through the natural history of unoperated congenital heart diseases, resulting in death during infancy and early childhood or major cardiovascular consequences in adulthood. The presentations in adults with VSD are variable, ranging from those with small VSD not considered for surgery in childhood, those operated in childhood with or without residual VSD, VSD with left-to-right shunt, and various degrees of left ventricular overload and pulmonary hypertension. Presentation with Eisenmenger syndrome remains a problem in sub-Saharan Africa. Echocardiographic findings in this case are consistent with a small VSD with an insignificant left-to-right shunt.
Symptoms in patients with VSD are mainly driven by left ventricular overload resulting in heart failure and increased pulmonary vascular resistance giving rise to pulmonary hypertension. These eventual consequences could result in variable combinations of shortness of breath, palpitation, cyanosis, hemoptysis, and rarely syncope. However, our patient had no features of increased loading conditions, and pulmonary pressure was normal. She presented with paroxysms of palpitation, shortness of breath, and near syncope with no limitations suggestive of cardiovascular compromise in the “interictal periods,” features reminiscent of paroxysmal SVT. This was confirmed on the ECG obtained at the ER.
The most common cardiac arrhythmias in VSD are premature ventricular contractions either in isolation, as couplets or multiform. Although some congenital cardiac anomalies, notably Ebstein's anomaly are associated with increased prevalence of accessory pathway-mediated tachyarrhythmias, paroxysmal SVT is not a common occurrence in VSD. Paroxysmal atrial tachyarrhythmia (not specified) was documented in 1% of adults with unrepaired VSD in the Belgian registry, while 4% of those with repaired VSD developed atrial flutter.
The presentation and findings in our patient did not fit any of the class I or II indications for the repair of VSD. Understandably, some authors have suggested the need for closure of restrictive VSD in adults differently from the recommendation in standard guidelines because of the high propensity of developing LV volume overload, pulmonary hypertension, and symptomatic heart failure in the future. A restrictive VSD without hemodynamic consequences in a patient with recurrent palpitation, shortness of breath, lightheadedness, and near syncope should prompt evaluation for cardiac arrhythmia including use of ambulatory ECG and/or electrophysiology study since the VSD in this instance may be a “red herring” unlikely to be culpable. In addition, the scar of open-heart surgery will undoubtedly create tissue heterogeneity and promote re-entry, predisposing the patient to other forms of supraventricular tachyarrhythmias including atrial fibrillation and atrial flutter.
| Conclusion|| |
This case underscores the importance of holistic evaluation of patients to determine the culprit lesion for a given presentation, especially where there is a discordance between clinical findings and structural abnormality. This will result in a more appropriate treatment and spare patient the costs and dangers associated with evaluation and treatment of “innocent” nonculprit lesions.
Although SVT was documented, Holter ECG would have been useful, given that other forms arrhythmias prevalent in SVD may be contributing to the patient's symptoms. Electrophysiology study was not done due to nonavailability in our center. Cardiac catheterization for assessment of shunts or cardiac MRI was not done. However, this may not be necessary since echocardiography did not show features suggestive of hemodynamically significant intracardiac shunts.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Abbott ME. Congenital heart disease. In: Nelson Loose-Leaf Medicine: A Perpetual System of Living Medicine. 4th
ed. New York: T Nelson & Sons; 1931.p. 207-321.
Perloff JK, editor. Ventricular septal defect. In: The Clinical Recognition of Congenital Heart Disease. 4th
ed. Philadelphia: WB Saunders; 1994. p. 396-439.
Martin GR, Perry LW, Ferencz C. Increased prevalence of ventricular septal defect: Epidemic or improved diagnosis. Pediatrics 1989;83:200-3.
Buba F, Talle MA, Anjorin CO, Baba MM, Ibrahim BA. Congenital heart diseases diagnosed on transthoracic echocardiography: Perspectives from the University of Maiduguri Teaching Hospital, Nigeria. World J Cardiovasc Surg 2017;7:55-65.
Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. N Engl J Med 2000;342:256-63.
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.
Baumgartner H, Bonhoeffer P, de Groot NM, de Haan F, Deanfield JE, Galie N, et al
. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010;31:2915-57.
Liberman L, Kaufman S, Alfayyadh M, Hordof AJ, Apfel HD. Noninvasive prediction of pulmonary artery pressure in patients with isolated ventricular septal defect. Pediatr Cardiol 2000;21:197-201.
Wolfe RR, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, Kidd L, et al
. Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring. Circulation 1993;87:I89-101.
Gabriels C, de Backer J, Pasquet A, Paelinck BP, Morissens M, Helsen F, et al
. Long-Term outcome of patients with perimembranous ventricular septal defect: Results from the Belgian Registry on Adult Congenital Heart Disease. Cardiology 2017;136:147-55.
Ammash NM, Warnes CA. Ventricular septal defects in adults. Ann Intern Med 2001;135:812-24.