Rodolfo Neirotti

Tetralogy of Fallot with Subpulmonary Ventricular Septal Defect

Among a total of 608 patients with tetralogy of Fallot, 35 with tetralogy and subpulmonary ventricular septal defect (VSD) have undergone intracardiac repair at the Childrens Hospital, Buenos Aires. The crista supraventricular was absent in 62.9% of these patients; it was hypoplastic in 37.1%. Only 3 patients (8.6%) were severely cyanotic and required operation in infancy. The main and left and right pulmonary arteries were relatively large in all but 1 patient. Nevertheless, patch-graft enlargement across the pulmonary valve ring was necessary in 20 (57.1%). We believe that closure of the subpulmonary VSD increase the apparent severity of the pulmonary stenosis and necessitates patch-graft enlargement in patients in whom the appearance of the right ventricular outflow tract before repair would not have suggested its need.

Unusual form of coarctation of the distal thoracic aorta

We report the case of a 3-month-old girl with a rare form of coarctation involving the lower descending thoracic aorta. Because of clinical findings of congestive heart failure and hypertension, early repair was recommended. Surgical intervention in young patients with this unusual localization presents a complex challenge. Aortic reconstruction was carried out by patching the stenotic segment with autologous arterial tissue. Three years after the repair, there is no evidence of recoarctation or aneurysmal dilation.

A massive pulmonary tumor embolism after surgical manipulation and biopsy of a pelvic mass.

The patient is a 3-yr-old, previously healthy boy who was noted to have fecal incontinence, difficulty and discomfort while sitting, and a burning sensation during urination. Physical examination suggested a pelvic mass. Computed tomography scans confirmed a large sacrococcygeal mass extending into the abdomen as well as metastatic disease in the lungs. Given the sacral involvement and its large size, it was thought that this tumor was unresectable; therefore, surgical biopsy was planned. An abdominal retroperitoneal approach was used because a future re-exploration was considered and was to be performed via a posterior sagittal approach. Usual monitoring was used. A 22-gauge peripheral IV was in situ, and anesthesia was induced with sodium pentothal, pancuronium, fentanyl, and isoflurane. The trachea was intubated, and esophageal temperature and breath sounds were monitored. Maintenance anesthesia consisted of isoflurane (1%) and N2O (50%) via a semiclosed circle system with supplemental fentanyl and pancuronium. After an abdominal incision, the patient was placed in Trendelenburg’s position, and the intestines were carefully retracted, exposing the retroperitoneum. Dissection revealed the tumor, extending toward the sacrum and retroperitoneal lymphadenopathy. The surgeon reported palpating a large vessel leading up to the mass, and a portion of the mass was felt to be within the lumen of the left internal iliac vein. Subsequent tissue biopsies were taken from an area adjacent to the sacral promontory. Inspection of the area near the large tumor mass in the vein revealed that the palpable mass was now gone. The surgeon informed the anesthesiologist about the possibility of venous embolism. There were no changes in blood pressure (85/52 mm Hg), heart rate (140 bpm), or electrocardiogram (sinus rhythm). Heart and lung sounds were unchanged. There was no heart murmur. Biopsies were sent to the pathologist for frozen section. Approximately 20 min later, the patient suddenly decompensated. The heart rhythm changed to frequent premature atrial contractions, premature ventricular contractions, bradycardia, and pulseless electrical activity. ETco2 decreased to 19 mm Hg, blood pressure decreased to 58/28 mm Hg, and oxygen saturation became undetectable. A massive tumor embolus was suspected. Fortunately, the surgery was performed in an operating room equipped for pediatric cardiac surgery, and transesophageal echocardiography (TEE) was immediately available. TEE demonstrated a grossly distended right atrium and right ventricle (RV). A large, foreign-body embolus was visualized migrating from the right atrium through the RV outflow tract to the main pulmonary artery. The RV was distended with a septal shift to the left, with compression of the left ventricle and global hypokinesis. The pediatric cardiovascular surgeon and perfusion team were notified for an attempt at tumor thrombus retrieval using cardiopulmonary bypass (CPB). Resuscitation took place for more than 40 min with cardiopulmonary resuscitation. Intermittent epinephrine and sodium bicarbonate boluses were administered. Pulseless electrical activity persisted until CPB was initiated. After a period of stabilization, tumor emboli were retrieved from the right and left pulmonary artery through a transverse incision in the main pulmonary artery. Small fragments of tumor were removed from the pulmonary artery branches with a Fogarty catheter. Initial arterial blood gas analysis and laboratory test values were pHa 7.31, Paco2 45 mm Hg, Pao2 216 mm Hg, HCO3 23 mEq/L, O2 saturation 100%, glucose 421 mg/dL, potassium 4.2 mEq/L, hematocrit 30%, ionized Ca 0.65 mmol/L, and lactate 18 mm Hg. After successful tumor emboli removal, a dobutamine infusion was started at 5 mg z kg z min. The TEE demonstrated good biventricular contractility with no wall motion abnormalities. Separation from CPB was uneventful. A left subclavian central venous pressure line was placed, and the patient was transported to the pediatric intensive care unit in a hemodynamically stable condition. Upon arrival to the pediatric care unit, the patient was responsive to auditory and tactile stimuli, occasionally opening his eyes, and moving all extremities. Mannitol was administered prophylactically for cerebral edema. The pathology report noted multiple fragments of tumor from the embolus measuring in aggregate 4 3 1 3 0.8 cm. The postoperative diagnosis was Stage IV malignant sacrococcygeal teratoma. The patient was discharged from the hospital 26 days after the initial operation. Neurologically, the patient experienced expressive aphasia and visual peripheral disturbances, which have subsequently resolved.