Case Report
A girl, who referred to our clinic with symtoms of congestive heart
failure, mild pulmonary hypertension (PAP : 30 mmHg) and resistant to
medical treatment. The patient was 3 monts old and 3.800 grams weight.
During the examination, S1 hard, S2 double, 3/6 pansystolic murmur was
heard in the lower left sternal region. The electrocardiogram was a
sinus rhythm at 150 beats /min and left axis deviation. Hemoglobin was
16.5 g/L and hematocrit was 52%. The patient with growt reterdation and
tachipnea has oxygen saturation 95 % at room air. On chest x-ray,
cardio-thoracic ratio was 0.6 and pulmonary plethora was seen. The
patient has no signs of Down’s syndrome.
In 2D transthoracic echocardiographic (TTE) examination, 7 mm primum ASD
in the interatrial septum and 5 mm VSD in the interventricular septum
were observed. It was observed that there was a second degree of
insufficiency in the left atrioventricular (AV) valve. Intracardiac
anomaly was named as AVSD transiotional type. At the base of the
interventricular septum, close proximity to membraneus septum, a
uniformly limited hyperechogenic mass of 1.5x1.0 cm was observed in the
membranous septum. This mass minimally protruding into the cavitiy of
right ventricle. (Fig.1) Doppler examination did not reveal any
evidence of inflow or ventricular outflow tract obstruction. PDA was
observed open and 6 mm in diameter. Cardiac mass seen by
echocardiography was evaluated in more detail with
multidetector
computed tomography (MDCT). (Fig.2)
The patient who was considered rhabdomyom was examined for advanced
tuberous sclerosis. With cerebral imaging, cortical tubers and
subependimal nodules were observed. Multiple renal kists were detected
by urinary ultrasonography. And after dermatological examination the
diagnosis of tuberous sclerosis became definitive. The patients
condition was discussed with pediatric cardiologists and surgical
intervention is planned.
The patient was taken to the operation room. Following the median
sternotomy, the thymus was excised subtotally and the pericardium was
opened vertically. Arterial cannulation from ascending aorta and venous
cannulations from both vena cava were performed. Double ligation and
division of the ductus arteriosus was performed. The cardiopulmonary
bypass (CPB) procedure was initiated. Cardioplegia was given from the
aortic root and diastolic arrest was achieved. Right atiotomy was
performed. Both primum ASD (7 mm) and secundum ASD (3mm) were observed
in IAS. Left atrial vent was put through secundum ASD. Atrioventricular
septal defect repair was done with using modified single patch tecnique.
On the right ventricular side of the IVS, on the membranous septum,
adjacent to the tricuspid valve anteroseptal comissures,
well-circumscribed, shiny surface, primary mass was detected.(Fig.3) In addition, bright, scattered lesions of millimeter
sizes were observed on the surface of the tricuspid valve. However, it
was not excised because the primary lesion was rhabdomyoma, asymptomatic
and did not cause functional and structural impairment. Additionally in
the case of resection atrio-ventricular block risk was high.
Subsequently, secundum ASD and right atriotomy was closed. Total CPB
duration was 57 minutes and cross clamp time was 43 minutes. When CPB
ended, the patient was in sinus rhythm. Dopamine supplementation at a
dose of 5 mcg / kg / min was started for the patient who needed
inotropic support in termination of CPB.
Inotropic support was discontinued at the 4th postoperative hour and
sedation support was discontinued at the 6th hour and extubated at the
8th hour. The patient was stayed in the intensive care unit for 22 hours
after extubation was taken to the inpatient ward. The patient was
followed up for 3 days in the ward and discharged on the 4th day with
healing. Control TTE was performed before discharge. It was observed
that IAS and IVS were intact and no color transition. It was confirmed
that the PDA was completely closed and there was no regurgitation of AV
valves.