Echocardiography of hypoplastic left heart syndrome

Abstract

Echocardiography is critical in the assessment of patients with hypoplastic left heart syndrome. Fundamental techniques and standardised approaches are useful when evaluating patients with hypoplastic left heart syndrome prenatally, after birth, and before the Norwood operation (Stage 1); after the Norwood operation, before and after the superior cavopulmonary anastomosis (Stage 2); before and after the Fontan operation (Stage 3); and for chronic surveillance after the Fontan operation. From foetal assessment to ongoing surveillance after the Fontan procedure, echocardiography remains the primary technique for cardiac monitoring in this growing population of children and adults.

Hybrid Palliation for Hypoplastic Left Heart Syndrome: Role of Echocardiography

“…There is great interest in prenatal diagnosis of HLHS, and the argument has been accurately discussed [ 21 , 22 , 23 ]. Although in the first trimester, the anatomy of the heart may not be well defined, as the only clue can be a hyperechogenic endocardial border of a near-normal left ventricle, in the second trimester, the anatomy appears extremely abnormal [ 23 ].…”

Perioperative Imaging in Hypoplastic Left Heart Syndrome

“…Because of the peculiar anatomy of the atrial septum in HLHS, the septum primum typically has a leftward and superior displacement of its insertion ( Figure 1). This particular anatomy results in an unusual location of the atrial communication 17 with a more superiorly and posteriorly directed jet across the atrial septum ( Figure 2). Therefore, it can be difficult to properly align the ultrasound beam with the flow across the atrial septum.…”

“…It is important to image the entire course of these decompressing veins with two-dimensional imaging and pulse wave Doppler, as they can become stenotic over time. 17 Patients with an intact or highly restrictive atrial septum without a decompressing vein require urgent intervention at birth. In utero, they have severely elevated left atrial and pulmonary venous pressure causing arterialization of the pulmonary veins and abnormal development of the entire pulmonary vasculature.…”

Resuscitation and Perioperative Management of the High-risk Single Ventricle Patient: First-stage Palliation

“…Optimizing Qp : Qs and DO 2 may involve mechanical ventilation, strategies to manipulate PVR (hypoventilation, inspired CO 2 , inspired nitrogen), augmentation of CO via inotropes, administration of agents to decrease SVR, or early surgical intervention prior to an expected postnatal decrease in PVR. Transthoracic echocardiography should be employed to assess patency of the ductus arteriosus, adequacy of the atrial level communication, myocardial function, tricuspid regurgitation, and the presence of other defects (such as anomalous pulmonary venous return) …”

“…Transthoracic echocardiography should be employed to assess patency of the ductus arteriosus, adequacy of the atrial level communication, myocardial function, tricuspid regurgitation, and the presence of other defects (such as anomalous pulmonary venous return). 57,58 PVR usually falls predictably during the first few days of life and initially manifests as comfortable tachypnea; over a variable time course, this may progress to congestive heart failure with cardiomegaly, pulmonary plethora, and hepatome-galy. As PBF continues to increase at the expense of SBF, a compensatory increase in CO occurs to maintain adequate DO 2 ; in extreme cases, impaired DO 2 may lead to multiorgan system failure.…”