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Case Reports
. 2022 May-Jun;15(3):308-310.
doi: 10.4103/apc.apc_181_21. Epub 2022 Nov 16.

An unusual cause of heart failure in postoperative tetralogy of Fallot

Gurbhej Singh  1 , Arun Gopalakrishnan  1 , Sivasubramonian Sivasankaran  1
Affiliations
Case Reports

An unusual cause of heart failure in postoperative tetralogy of Fallot

Gurbhej Singh et al. Ann Pediatr Cardiol. 2022 May-Jun.

Abstract

The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etiology, 28 years after TOF repair. Cardiac catheterization confirmed findings of elevated right heart pressures. Magnetic resonance imaging showed obliteration of the RV apex with late gadolinium enhancement of the right ventricular apical endomyocardium.

Keywords: Congenital heart disease; Tetralogy of Fallot; echocardiography; endomyocardial fibrosis; heart failure.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Continuous wave Doppler across ventricular septal defect showing left-to-right shunt (red arrow) during systole and right-to-left shunt during diastole (yellow arrow). (b) Color M-Mode shows the left-to-right shunt (red arrow) during systole and right-to-left shunt during diastole (yellow arrow). (c) Magnetic resonance image showing RV apical obliteration (Blue arrow). (d) Magnetic resonance imaging showing late gadolinium enhancement of RV apex endomyocardium (Blue arrow). RV: right ventricular
Figure 2
Figure 2
(a) RV angiogram (RAO 30°) during diastole shows apical obliteration and diverticuli resembling a caterpillar. (b) RV angiogram during systole shows disappearances of diverticuli. (c) LV angiogram in RAO view shows no involvement of left ventricle and site of VSD jet (red arrow). (d) Simultaneous left ventricular and right ventricular pressure traces showing RV ed 17 mmHg, LV ed 10 mmHg. LV ed – Left ventricular end-diastolic pressure, RV ed – Right ventricle end diastolic pressure. RV: Right ventricular, RAO: right anterior oblique, LV: Left ventricular, VSD: Ventricular septal defect
See this image and copyright information in PMC

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