Case presentation
A female infant with a birth weight of 2,231 g was delivered vaginally
at a gestational age of 37 weeks and 6 days. A fetal echocardiogram
revealed a heart abnormality with severe aortic and pulmonary stenosis.
Tracheobronchial tree and lung abnormality were not reported in fetal
diagnosis. After birth, she was tachydyspneic and cyanotic with a loud
“to-and-fro” murmur over the left sternal border. Hepatomegaly was
observed. Chest radiography revealed a cardiothoracic ratio of 80%.
Echocardiography showed the absence of pulmonary valve with pulmonary
insufficiency and stenosis, L-TGA, dextrocardia, double outlet left
ventricle, hypoplastic anatomical right ventricle, valvular aortic
stenosis, atrial septal defect, and patent ductus arteriosus with a
bidirectional shunt. Computed tomography (CT) showed
a severe hypertrophic anatomical
left ventricle connected to the left displaced and dilated main
pulmonary artery, with dilation of both branches (Figure 1). The
compression of the left main bronchus by the dilated pulmonary artery
was diagnosed as tracheomalacia (Figure 2A). The left lung was
compressed by the enlarged heart and was suspected to be hypoplastic. It
had a less vascular shadow than the right lung.
A tracheal tube was placed immediately after birth because the
spontaneous breathing effort was severe, and left lung sounds could not
be heard on auscultation. A Ventricular arrhythmia requiring adrenalin
infusion immediately after birth indicated severe heart failure. Six
hours after delivery, the patent ductus arteriosus was ligated because
of increased pulmonary insufficiency and exacerbation of heart failure.
On postnatal day 5, percutaneous balloon aortic valvuloplasty was
performed for critical aortic valve stenosis. This reduced the peak
pressure gradient from 25 mmHg to 20 mmHg. At 4 months age, anatomical
left ventricle to pulmonary artery shunt using an 8-mm expanded
polytetrafluoroethylene bicuspid-valved conduit with bulging sinuses
(Stretch Vascular Graft, W.L. Gore & Associates, Inc., Flagstaff, AZ,
USA) and pulmonary artery plication were performed. Although pulmonary
insufficiency could be controlled completely with the operation,
tracheomalacia of the left main bronchus remained (Figure 2B), and a
high positive ventilation setting was still required after the
operation. Tracheostomy was performed at the age of five months. There
was poor postoperative weight gain and repeated necrotizing
enterocolitis. Cartelization showed that her Qp:Qs was 1.6:1, and
aortopulmonary collaterals were observed from the right intrathoracic
artery. Embolization of the aortopulmonary collateral was performed to
decrease the pulmonary blood flow at the age of nine months. Conduit was
clipped at the age of ten months to decrease pulmonary blood flow. There
was good weight gain, and positive ventilation was not required after
the surgery, although tracheomalacia of the left main bronchus persisted
on CT. Despite recurrent respiratory tract infections, the patient was
discharged at the age of 14 months in a stable condition while waiting
for the Glenn procedure until
getting her parent’s permission.