Hypoplastic left heart syndrome: Hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified fontan procedure

Abstract

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.

Impact of Re-Coarctation Following the Norwood Operation on Survival in the Balloon Angioplasty Era

“…Re-coarctation is common in patients who have undergone a Norwood operation for hypoplastic left heart syndrome (HLHS), with a reported incidence from 11% to 37% (1)(2)(3)(4)(5)(6)(7). After the efficacy of balloon angioplasty (BA) was demonstrated for native and postoperative aortic arch obstruction, the procedure was applied to patients with HLHS (8 -12).…”

Real-Time 3-Dimensional Echocardiography Provides New Insight Into Mechanisms of Tricuspid Valve Regurgitation in Patients With Hypoplastic Left Heart Syndrome

“…3,4 In general, TR may be due to dilatation of the right ventricle (RV), RV geometrical change, left ventricle (LV)-RV interaction, RV dysfunction, and intrinsic abnormalities of the leaflets and supporting apparatus. [1][2][3][4] Several studies have attempted to address risk factors for the development of TR [2][3][4][5] but have provided little insight.…”

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

“…From our studies evaluating preoperative catheterization results, only moderate or severe tricuspid valve regurgitation after initial reconstructive surgery appears to predict poor outcome following a Fontan procedure. 17 This relatively homogeneous study population is younger than those described in most previous studies of patients undergoing a modified Fontan procedure. ",'2 Alternative surgical strategies for young children considered to be at high risk for a Fontan procedure as primary therapy have been proposed, including the bidirectional cavopulmonary anastomosis, [18][19][20] …”