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AORTO-LEFT VENTRICULAR TUNNEL – FETAL DIAGNOSIS AND EARLY NEONATAL TREATMENT AS A PRECONDITION FOR SUCCESFUL DISEASE OUTCOME
IVAN MALČIĆ
; Zagreb University Hospital Centre, Department of Pediatric Cardiology, Zagreb, Croatia
DIJANA GRIZELJ KALČIĆ
; Dubrovnik General Hospital, Department of Pediatrics, Dubrovnik, Croatia
OLIVER VASILJ
; Sveti Duh University Hospital, Department of Obstetrics and Gynecology, Zagreb, Croatia
MAJA HRABAK PAAR
; Zagreb University Hospital Centre, Department of Diagnostic and Interventional Radiology, Zagreb, Croatia
Abstract
We present a patient with aorto-left ventricular tunnel (ALVT) diagnosed in the gestational age (GA) of 27 weeks. Examination was indicated by an obstetrician due to cardiomegaly. ALVT manifested itself by an extreme dilatation of the left ventricle (LVIDd 32 mm, >2 SD), a signifi cantly decreased contractile ability (ejection fraction (EF) 40%) and color Doppler display of an asymmetric aortic valve insuffi ciency via a canal fl owing from the sinotubular junction to the left ventricle, between the interventricular septum and subinfundibular portion of the right ventricle. Myocardial performance index was 0.62 (normal 0.25-0-50), cardiovascular profi le score 4 (normal 10), a-wave in ductus venosus (DV) positive (++) (as positive evidence for fetal cardiac dysfunction) and positive umbilical venous pulsations. In addition, an extremely dilated tubular aorta was also found (18 mm – >2 SD, above 95th percentile). In that gestational age, aortic valve ring was measured 5.8 mm (75th percentile), and the width of extravalvular tunnel 4-5 mm. Since no hydrops was found, in utero treatment with medicines was not used. Cardiomegaly was found postpartum (x-ray), and the ALVT diagnosis was confi rmed by echocardiography. The canal was located above the right coronary cusp, i.e. in front of the raphe connecting the left and right coronary cusp, meaning that the diagnosis of a bicuspid aortic valve (BAV) of anterior-posterior orientation was also made. Inotropic and diuretic therapy was ordered due to cardiac insuffi ciency development. Diagnosis was also confi rmed with multi-slice computed tomography and heart catheterization (angiocardiography). Cardiac surgery was performed at the age of 16 days by placing an autologous pericardial patch on the aortic canal opening. Diuretic, digitalis and angiotensin-converting enzyme inhibitor were ordered postnatally. Left ventricle was recovering incrementally (3 months after surgery, EF 50%), but pathological dilatation of tubular aorta persisted. Complete recovery of the left ventricle is expected, with the need for further observation of the patient because of the BAV and possible effects on the valve itself and on the ascending aorta. At the age of 10 months, contractility of the left ventricle was normal (EF 65%), with mild aortic insuffi ciency (central), normal fl ow through the aortic valve, but dilatation of the ascending aorta persisted (ascending aorta width 2.1 cm, >2 SD).
Keywords
aorto-left ventricular tunnel; prenatal diagnosis; cardiac insuffi ciency; early cardiosurgical therapy; fetal cardiology; rare diseases
Hrčak ID:
244560
URI
Publication date:
6.10.2020.
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