Gerbode defect formation two decades after tetrology of fallot repair

Abstract

Although most intracardiac defects are congenital, a small fraction may be acquired during life. The Gerbode defect is an abnormal anatomical connection between the left ventricle and the right atrium. We describe herein a patient who initially underwent repair of tetralogy of Fallot (TOF). Years after TOF repair, he developed severe dyspnea. Extensive evaluation revealed that he had developed a Gerbode defect. Very few cases of acquired Gerbode defect have been previously reported. Management options are predominantly surgical interventions.

Congenital Gerbode Defect: A Left Ventricular to Right Atrial Shunt—State-of-the-Art Review of Its General Data, Diagnostic Modalities, and Treatment Strategies

“…In addition, the increased right ventricular preload causes pulmonary circulation overload with a subsequent volume loading and enlargement of the left heart cavities [ 1 , 33 , 38 ]. Thus, in the case of a significant shunt with therapeutic delay, the acyanotic Gerbode defect [ 44 ] may result in dilatation of all cardiac cavities, pulmonary artery hypertension and congestion, as well as low cardiac output and a decline in global cardiac function [ 11 , 50 ].…”

“…In addition, a prolonged P-R interval and peaked right atrial P waves indicate right atrial dilatation [ 26 ]. Atrial fibrillation may also occur [ 11 ].…”

“…In addition to transthoracic echocardiography, transesophageal echocardiography (TEE) is an elementary mainstay of diagnostics featuring higher preciseness and repre-senting the most sensitive diagnostic modality for assessing the Gerbode defect [ 11 , 12 , 53 , 54 , 55 ]. According to the literature review by Yuan, TEE was superior to TTE in terms of diagnostic accuracy, rate of missed diagnoses, and rate of inclusive diagnoses, whereas misdiagnosis rates did not differ between TTE and TEE [ 5 ].…”

“…Possible treatment options include conservative monitoring, cardiac surgery, or interventional device closure [ 37 ]. The selection of individual therapy should be based on the presence of chamber enlargement and the severity of symptoms, which depends on factors such as the size, location, and magnitude of the shunt, flow volume, and concomitant cardiac abnormalities [ 1 , 11 , 67 ]. Consequently, asymptomatic patients presenting with an insignificant small LV-RA shunt, without circulatory overload, may be managed by conservative follow-up without accessing surgical closure [ 1 ].…”

“…However, this systematic review of the literature focuses on the congenital form of the Gerbode defect. Although congenital LV-to-RA shunt represents a rare disease, accounting for less than 1% of all congenital cardiac defects [ 1 , 5 , 7 , 8 , 9 , 10 , 11 ], it has significant clinical importance [ 12 ]. Various comorbidities and complications that may occur during the course of disease have a considerable impact on the outcome.…”