Designing therapeutic strategies for patients with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs

Abstract

The palliation of the cyanotic child with a dominant morphologically left ventricle, discordant ventriculo-arterial connections, and obstruction to the pulmonary outflow tract has continued to evolve and mature. The evolution began in the early days of surgical palliation with the Blalock-Taussig shunt, extended to construction of cavopulmonary shunts, if required, and then to the Fontan procedure and its subsequent modifications. This journey took nearly 30 years to complete. There is increasing clinical data to document the beneficial effects of this approach, with ever-improving outcomes. Some aspects of the history of the cavopulmonary shunt have been previously reviewed in this journal and elsewhere, as have analysis of outcomes for some groups of patients considered for surgical completion of the Fontan circulation. While there has been some ongoing interest in ventricular septation since the early success of Sakakibara et al., this approach has largely been abandoned. Considerably more challenges and debate resonate in the surgical algorithms defined for patients whose hearts are characterized by a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs. This latter group will be the focus of this review, as will the aetiology of the myocardial hypertrophy that is particularly frequent in this group of patients, its clinical recognition, indeed its anticipation, and the multiple surgical strategies designed to prevent or treat it. All these manoeuvres are considered to optimise suitability for, and outcome from, creation of the Fontan circulation.

Arterial Switch, Ventricular Septation, and Fontan Takedown for Double Inlet Left Ventricle

“…However, since the Fontan-type single ventricular reconstruction was developed, Fontan reconstruction has become the standard treatment for DILV. Ventricular septation has only been reported in several centers owing to its higher surgical complexity and mortality [2,3]. Although Fontan circulation still has some limitations, favorable long-term results after ventricular septation have been reported [4], with better cardiopulmonary function compared with Fontan circulation [3].…”

“…Systemic outflow obstruction jeopardizes the longterm outcome of DILV [1]. Subaortic stenosis can be present at birth or develop later in life [2]. For subaortic stenosis in DILV, there are several surgical choices, e160 HOW TO DO IT including myomectomy only, Damus-Kaye-Stansel procedure, palliative arterial switch, and others [2].…”

“…Subaortic stenosis can be present at birth or develop later in life [2]. For subaortic stenosis in DILV, there are several surgical choices, e160 HOW TO DO IT including myomectomy only, Damus-Kaye-Stansel procedure, palliative arterial switch, and others [2]. We decided to perform a biventricular repair for the patient because the pulmonary valve regurgitation will become aortic regurgitation if the Damus-Kaye-Stansel procedure is performed; and because the patient's subaortic stenosis, right pulmonary artery stenosis, and atrioventricular valve regurgitation would possibly compromise the long-term results of Fontan circulation.…”

“…(Ann Thorac Surg 2018;106:e159-62) Ó 2018 by The Society of Thoracic Surgeons S ystemic outflow obstruction compromises heart function in patients with double-inlet left ventricle (DILV) [1]. Surgical options for subaortic stenosis included enlargement of the ventricular septal defect, the Damus-Kaye-Stansel procedure, and valved conduit from the apex [2]. Herein we present a new surgical option for DILV with transposition of great arteries (TGA) and severe subaortic stenosis after total cavopulmonary connection (TCPC).…”

Prediction of Systemic-to-Pulmonary Artery shunt surgery outcomes using administrative data

“…The Systemic-to-Pulmonary Artery (SPA) shunt is a common procedure, often performed in the neonatal period, used to establish a reliable source of pulmonary blood flow in babies with limited or absent pulmonary blood flow [8,15]. It is also often an integral part of staged palliative procedures for selected neonates and infants with complex heart defects.…”