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Washington University Experience | VASCULAR | Hemorrhage - Neonatal | 9A0 Case 9 History
Case 9 History ---- The patient was a 2500 gm, 39 week EGA female born on 8/25 to a G2Pl mother by repeat cesarean section. Pregnancy was uncomplicated. Apgars were 7 at 1 minute and 5 at 5 minutes. The patient was noted to have a heart murmur and was cyanotic despite good respiratory effort, with an ABG on 80% oxygen of pH7.35/pC02 34/p02 43/HC03 19. The patient was transferred to SLCH where she was still cyanotic and chest X-ray showed an enlarged heart with an increase in right ventricular size. Initial evaluation demonstrated that the cyanosis was not affected by supplementary oxygen administration, consistent with cyanotic congenital heart disease. Echocardiogram showed Tetralogy of Fallot versus double outlet right ventricle (DORV) with pulmonic stenosis, a small patent ductus arteriosus (PDA) and an atrial septa! defect. Family history revealed velo-cardio-facial (VCF) syndrome in the patient's mother and 3 year old brother, neither of whom had any heart defects. The initial hospital course was complicated by persistent hypoglycemia which was believed due to congenital heart disease. The patient was also found to have a submucosal cleft involving the secondary palate, consistent with the VCF syndrome. By 8/30, oxygen saturations showed a declining trend, with 74-80% saturation and decreases to 60% with minimal movement, so the patient was scheduled for Blalock-Taussig (B-T) shunt placement. Cardiac catheterization on 8/31 revealed DORV with a large ventricular septal defect, pulmonic valvular and infundibular stenosis, mild aortic stenosis, no PDA, a right sided aortic arch and systemic right ventricular pressure. Catheterization was complicated by respiratory arrest requiring transfer to the PICU. On 9/1, the patient was brought to the OR for B-T shunt placement. The patient sustained a severe cyanotic episode with need for high dose pressor/inotropic resuscitation. Due to the patient's critical condition and operative difficulty, a central shunt was placed from the aorta to the right pulmonary artery. Post-operative course s complicated by refractory metabolic acidosis and oliguria secondary to severe biventricular failure and the patient was placed on ECMO. Ventricular function gradually recovered and the patient was decannulated from ECMO without difficulty on 9/5. Later on 9/5, the patient abruptly became bradycardic to 70 bpm and hypotensive with systolic pressure of 30. CPR and pharmacologic agents were begun; approximately 15 minutes into resuscitation the chest tube drained 50 cc of blood. Resuscitation was unsuccessful and the patient expired on 9/5. ---- At autopsy, examination of the brain revealed clotted blood in both right and left CNS ventricles. Microscopic examination revealed that most of this blood was associated with the choroid plexus which contained markedly dilated vessels and interstitial hemorrhage, which is often the history in choroid plexus histories. In addition, there was a small subependymal hemorrhage in the left lateral ventricle, just caudal to the foremen of Monro. This is consistent with the clinically documented left subependymal hemorrhage noted on ultrasound.
