Fetal Aortic Valve Stenosis and the Evolution of Hypoplastic Left Heart Syndrome

Abstract

Background-Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS. Methods and Results-Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at Յ30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS. Conclusions-In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.

Prenatal detection of congenital heart disease—results of a national screening programme

“…7,[10][11][12][13][14] Prenatal detection of specific types of CHD may reduce neonatal mortality and morbidity. [15][16][17][18][19] It allows for planning the delivery at a tertiary-care centre ensuring optimal neonatal and perisurgical care. Furthermore, parents can consider termination of pregnancy (TOP) in severe cases.…”

Clinical characteristics and outcomes in patients with endocardial fibroelastosis associated with critical aortic stenosis with biventricular circulation

“…In a study by Mäkikallio et al, [19] of the 23 patients diagnosed with aortic stenosis who had a normal left ventricular length at ≤ the 30 th gestational week without fetal intervention, only six had biventricular circulation. In addition, they found that a retrograde flow in the transverse aortic arch, left-to-right shunting across the foramen ovale, a monophasic mitral inflow, and significant left ventricular dysfunction were risk factors for the progression to HLHS.…”

Cirurgia fetal no contexto atual

“…Um grupo do Children's Hospital de Boston estabeleceu critérios bem definidos para indicação do procedimento, com o objetivo de encontrar aquelas pacientes com evolução evidente para hipoplasia do coração esquerdo, mas ainda em estágio reversível, obtendo a melhor relação risco-benefício 62 .…”