Unusual aortic valve anomaly in the fetus: a case report.

Aortic valve anomalies in fetal life usually concern aortic valve stenosis, in severe forms associated to left ventricular impairment - endocardial fibroelastosis and mitral valve insufficiency. Isolated aortic regurgitation in utero is infrequent and is usually considered to be due to a rare anomaly: aorto-left ventricular tunnel. We describe an unusual case of fetal aortic valve anomaly with severe dysplasia, with a marked regurgitant flow through the aortic valve, passing in a retrograde way from the duct, associated with a marked left ventricular endocardial fibroelastosis and dysfunction, resulting in the fatal outcome of the case.

Early Doppler-echocardiography evaluation of Carpentier-Edwards Standard and Carpentier-Edwards Magna aortic prosthetic valve: comparison of hemodynamic performance.

OBJECTIVES: This study was designed to describe Doppler-echocardiography values of Carpentier-Edwards Perimount Standard (CEPS) and Carpentier-Edwards Perimount Magna (CEPM) aortic prosthetic valves, evaluated by a single, experienced echo-laboratory, early in the postoperative phase. METHODS: Three-hundred-seventy-seven consecutive patients, who had had a CEPS or a CEPM implanted in our Hospital due to aortic stenosis and/or insufficiency, underwent baseline Doppler echocardiography evaluation within 7 days after surgery. Hemodynamic performances of CEPS and CEPM were accurately described, evaluating flow-dependent (transprosthetic velocities and gradients) and flow-independent (effective orifice area, indexed effective orifice area and Doppler velocity index) Doppler-echocardiography parameters. RESULTS: Out of the 377 patients 48.8% were men (n = 184), mean age was 74.63 ± 6.77 years, mean BSA was 1.78 ± 0.18 m2, mean ejection fraction was 57.78 ± 8%. Two-hundred and sixty two CEPS and 115 CEPM were implanted. Comparing size-by-size CEPS with CEPM, both prostheses showed a good hemodynamic profile, with fairly similar values of pressure gradients (PGmax and mean, in mmHg, = 37,18 ± 11.57 and 20.81 ± 7.44 in CEPS n°19 compared to 32,47 ± 7,76 and 17,67 ± 4.63 in CEPM n°19 and progressively lower in higher sized prostheses, having PGmax and mean 15 ± 3,16 and 9.15 ± 1,29 in CEPS n°29 compared to 15,67 ± 1,53 and 9 ± 1 in CEPM n°29) and EOAi (being 0,65 ± 0,33 cm²/m² in CEPS n°19 compared to 0,77 ± 0,29 cm²/m² in CEPM n°19 and progressively higher in higher sized prostheses, being 1,28 ± 0,59 cm²/m² in CEPS n°29 compared to 1,07 ± 0,18 cm²/m² in CEPM n°29), the latter resulting, however, basically less flow obstructive. CONCLUSIONS: Our data confirm the good hemodynamic performance of both aortic bioprostheses and the more favourable hemodynamic profile of CEPM compared to CEPS, pointing out the need to perform routinely an accurate baseline Doppler-echocardiography evaluation early after surgery to allow an adequate interpretation of data at follow-up.

Bicuspid aortic valve: theoretical and clinical aspects of concomitant ascending aorta replacement.

Bicuspid aortic valve (BAV) is associated with annuloaortic ectasia, dissection and ascending aortic aneurysm. The high incidence of this congenital malformation and aortic disease suggests a close correlation between the two phenomena. Abnormalities in different phases of cell migration of the neural crest might be responsible for the occurrence of abnormalities in the aortic valve, media layer of the ascending aorta and vessels of the aortic arch. Previous studies have shown that patients with normal BAV or slight dysfunction may present with dilation of the aortic root. The hemodynamic changes caused by BAV without stenosis or insufficiency seem to be an insufficient explanation for these findings. Several mechanisms have been proposed to explain the molecular and hystological aspects of this disease. We found a reduced fibrillin-1 content in both ascending aorta and pulmonary trunk as a possible cause. Histologically, the ascending aorta can present cystic medial necrosis and elastic fragmentation, similar to Marfan's disease. Some authors concluded that many patients, mainly those with aortic regurgitation, should have the aortic valve and the ascending aorta replaced at the same procedure, even if a mild dilatation (45 mm) is present in patients with BAV if life expectancy is anticipated to be greater than 10 years to prevent further aneurysms or ruptures.

Valva aórtica bicúspide: fundamentos teóricos e clínicos para substituição simultânea da aorta ascendente / Bicuspid aortic valve: theoretical and clinical aspects of concomitant ascending aorta replacement

A valva aórtica bicúspide (VAB) está associada à ectasia ânulo-aórtica, aneurisma e dissecção da aorta ascendente. A alta incidência desta malformação congênita e doença da aorta sugere íntima relação entre os fenômenos. Anormalidades ocorrendo em diferentes fases da migração das células da crista neural podem ser responsáveis pela ocorrência em anormalidades na valva aórtica, na camada média da aorta ascendente e nos vasos do arco aórtico. Estudos prévios revelam que mesmo indivíduos com VAB normal ou com disfunção leve podem apresentar dilatação da raiz aórtica. Os autores acreditam que somente as alterações hemodinâmicas produzidas por uma VAB sem estenose ou insuficiência parecem ser insuficientes para as graves complicações vasculares observadas nos portadores de VAB. Vários mecanismos têm sido propostos para explicar os achados moleculares e histológicos desta doença. Encontramos a redução da fibrilina-1 na aorta ascendente e artéria pulmonar como possível causa. Histologicamente, a aorta ascendente pode apresentar necrose cística da média e fragmentação elástica, semelhante àquela encontrada em portadores de síndrome de Marfan. Vários autores atualmente recomendam que em se operando um paciente com VAB, especialmente aqueles com insuficiência aórtica, mesmo na presença de uma discreta dilatação (45 mm) deve-se substituir a valva aórtica e a aorta ascendente concomitantemente quando a expectativa de vida exceder 10 anos, para se prevenir futuros aneurismas ou rupturas.

Bicuspid aortic valve (BAV) is associated with annuloaortic ectasia, dissection and ascending aortic aneurysm. The high incidence of this congenital malformation and aortic disease suggests a close correlation between the two phenomena. Abnormalities in different phases of cell migration of the neural crest might be responsible for the occurrence of abnormalities in the aortic valve, media layer of the ascending aorta and vessels of the aortic arch. Previous studies have shown that patients with normal BAV or slight dysfunction may present with dilation of the aortic root. The hemodynamic changes caused by BAV without stenosis or insufficiency seem to be an insufficient explanation for these findings. Several mechanisms have been proposed to explain the molecular and hystological aspects of this disease. We found a reduced fibrillin-1 content in both ascending aorta and pulmonary trunk as a possible cause. Histologically, the ascending aorta can present cystic medial necrosis and elastic fragmentation, similar to Marfan's disease. Some authors concluded that many patients, mainly those with aortic regurgitation, should have the aortic valve and the ascending aorta replaced at the same procedure, even if a mild dilatation (45 mm) is present in patients with BAV if life expectancy is anticipated to be greater than 10 years to prevent further aneurysms or ruptures.