7-year-old girl with tetralogy of Fallot and agenesis of the pulmonary valve

Clinical data
Patient presented a hypoxic in the neonatal period, reason for the early indication of a systemicpulmonary anastomosis with 13 days of life. Fatigue from birth did not prevent him from
performing frank physical activity. It remained with discrete cyanosis, hardly to the efforts,
although Blalock-Taussig was not observed patent since a year of age. She has been using
digoxin since she was three.
Upon physical examination, the patient presented good general condition, eupneic, acyanotic
and with normal pulses. The arterial saturation was 88.0% in wakefulness and the aorta was not
palpated in the furcula.
In the precordium, there were discrete pulses in the left sternal border and the stroke was
palpated in the 4th left intercostal space. The cardiac sounds were hyperphonectic and systolic
and diastolic murmur, + / ++ intensity, were heard along the left sternal border, without thrill.
The liver was not palpated and the lungs were clean, without adventitious noises.
The electrocardiogram showed sinus rhythm and signs of biventricular overload with
predominance of the right ventricle. AQRS was at + 90 , AP: + 60 , AT: + 80

Radiographic image
It shows the slightly enlarged cardiac area with rounded ventricular arch, middle bowed arch ++
+ and increased pulmonary vascularization, mainly in pulmonary hilios

Diagnostic impression
The image is compatible with the diagnosis of
pulmonary valvular insufficiency, which causes
large dilatations of the main pulmonary arteries. In
general, this anomaly accompanies the tetralogy of
Fallot with pulmonary annular stenosis and
ventricular septal defect.
Differential diagnosis
Other heart diseases associated with pulmonary
valve insufficiency may also manifest in the aforementioned manner. These include valvular

pulmonary stenosis, heart disease with dilated pulmonary arteries due to marked pulmonary
hyperflow, and even pulmonary arterial hypertension. All in a long evolution.
Diagnostic confirmation
The clinical features were decisive for the diagnosis of tetralogy of Fallot with pulmonary valve
agenesis, with a systolic-diastolic murmur with discrete hypoxemia (bidirectional shunt due to
VSD), and mainly due to the characteristic radiographic image.
The echocardiogram confirmed this diagnosis with the pulmonary arteries being very dilated
with diameters of 24 mm on the right and 14 mm on the left. The pulmonary ring was narrow
with 8 mm, in addition to infundibular pulmonary stenosis with anterior diversion of the
infundibular septum and large subaortic IVC, 15 mm in diameter. There was pulmonary valve
insufficiency and even insufficiency of the tricuspid valve. The ventricles were dilated

Cardiac catheterization showed similar pressures in both ventricles (80/10 mmHg) and
pulmonary trunk pressure (30/5 mmHg) showed marked pulmonary valve insufficiency, in
addition to valvular stenosis.
Conduct
At surgery, in the ECC of 160 'and anoxic period of 70', the infundibular stenosis was resected
by the right atrium and, through the pulmonary trunk, the agenesis of the pulmonary valve was
visualized with remnants, thick and retracted, ring Pulmonary function. The RV outflow tract
was enlarged for the pulmonary trunk and the IVC closed with bovine pericardium. Homograft
number 18 decellularized was implanted in pulmonary position and tricuspid insufficiency
corrected with annuloplasty.

The patient evolved well and was discharged after 7 days.

Team.
Hediberto Anza Alvarez
Kehiry Angelica Guillen Dominguez
Tania Alondra Cundapi
Mayra Lugo Cura