A 10-day-old male neonate with birth bodyweight of 3700 g, firstborn of non-relative parents in which the pregnancy was induced by intrauterine insemination (IUI) and the baby was born by cesarian section (C/S) with a gestational age of 40 weeks and good Apgar score was referred to the pediatric emergency department of Motahari hospital of Urmia. According to the mother's statements, the chief complaints were shortness of breath, rapid breathing, and vomiting. Also, the patient had a history of NICU admission on 2nd day after birth because of dehydration and poor feeding for 1st day. The patient could not take enough oral feeding for the last 2 days and vomiting was progressive.
On physical examinations, the anthropometric measures were normal, the patient was non-dysmorphic and chest X-ray showed mild to moderate cardiomegaly with bilateral perihilar infiltrates. Vital signs were as follows; PR: 162/min, RR: 62/min, BT: 36.5 C, and oxygen saturation of 94% (room air). Mucous membranes were dry but skin turgor and fontanels were normal. The chest shape was normal and symmetric with subcostal retraction and tachypnea. Cardiac auscultation revealed a grade II/VI continuous murmur. Peripheral pulses were palpable. On abdominal examination, the liver span was increased on palpitation and generalized edema was obvious. Primitive neonatal reflexes such as Moro, sucking, and rooting were normal.
The urgent ABG evaluation revealed PH of 7.14, PaCO2 of 29 mmHg, HCO3 of 9 mEq/L, and PaO2 of 43 mmHg. Serum electrolytes, BUN, creatinine, serum albumin, and blood sugar were in normal ranges. Primary complete blood count (CBC) and coagulation tests were normal except for the platelet count and INR. The patient had thrombocytopenia (platelet count of 95x103/mm3) and an INR of 1.19.
Due to severe pulmonary distress, the patient was intubated and admitted to NICU. After stabilizing the vital signs and hydration, a pediatric cardiology consultation was done. Echocardiography demonstrated no visible RPA, PDA with right to left flow, mild supravalvular aortic stenosis, mild aortic insufficiency, a severe reversal flow in aortic arch, secundum type ASD, dilated right atrium, right ventricle, and main pulmonary artery, severe tricuspid regurgitation and severe pulmonary hypertension, moderate mitral regurgitation and pulmonary insufficiency, dilated inferior vena cava, mild bilateral pleural effusion, and severe ascites. Due to the findings, close cardiological follow up and CT-angiography was performed.
Multi-slice computed tomography of the heart and major vasculature with special reconstructed views (dynamic and delayed images with contrast medium) revealed; anomalous arising of RPA from ascending aorta (hemitruncus), PDA 3.5 mm in size, stretched PFO (6 mm), atelectasis and congestion in both lungs with pleural effusion (Figure 1).
Serious efforts to perform cardiac surgery in addition to medical therapy such as inotropes and diuretics began. However, pediatric cardiac surgery was not available in the health care center or nearby cities. So, the patient was maintained on medical therapy and supportive care until cardiac surgery was a possibility.
Gradually, the patient's respiratory distress increased and liver function tests were more impaired and eventually cardiopulmonary arrest occurred on the 24th day of admission in the NICU. Immediate CPR was begun by cardiac massage, intravenous epinephrine, and positive pressure ventilation. After 30 mins the patient had no vital response and CPR was stopped. The last blood tests revealed metabolic and respiratory acidosis. The last blood test values were in Table 1.