Systemic ventricular dysfunction when things get mixed up

Introduction

Congenitally corrected transposition of great arteries (ccTGA) is a relatively rare cardiopathy, occurring in 1 case in 33 000 live births. It is rarely isolated and is often associated with other anomalies such as ventricular septal defects (80% cases), tricuspid valve anomalies (70% cases), pulmonary tract obstructions (30–50% cases), and sub aortic tract obstructions (rarely). However, association with coarctation of the aorta remains fairly rare, with prevalence poorly defined in the literature. Conduction disorders are frequent due to the existence of two AV nodes, with an annual risk of AVB of 2%.

Objective

To describe a clinical case associating ccTGA with a coarctation of the aorta revealed by a complete AV block.

Methods

We report the case of a 41-year-old man, hypertensive on dual antihypertensive therapy with poorly controlled hypertension, presenting with syncope and stage II NYHA dyspnea. The EKG showed complete AV block Transthoracic echocardiography showed double atrioventricular and ventriculoarterial discordance, situs solitus,levocardia, severe systemic right ventricular dysfunction and severe coarctation of the aorta. A thoracic CT scan confirmed coarctation of the aorta (Figure 1).

Expected results

The AV block and coarctation of aorta were considered to be the two main factors in systemic ventricular dysfunction.

It was decided to implant the patient with ventricular resynchronization (CRT-P), while the coarctation of the aorta was treated percutaneously.

Perspectives

Systemic ventricular failure should raise the suspicion of pressure overload, such as coarctation of the aorta. Early removal of the obstruction can delay deterioration of the contractile function of the systemic ventricle.