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.