There was moderate degree of tricuspid regurgitation, and the estimated pulmonary artery pressure was found to be 65 mmHg after calculation of the right atrial pressure from the inferior venae cava size and variation with respiration. Situs solitus, levocardia, with normal connections, normal biventricular sizes and systolic functions, normal left-sided cardiac valves appearances with mild mitral regurgitation, with tricuspid aortic valve showing trivial aortic regurgitation. Neonatal sepsis was the first differential diagnosis with septic shock, and other differential diagnoses are severe aortic coarcatation with duct-dependent systemic circulation and aortic interruption. Laboratory investigations including blood cultures were withdrawn immediately, the patient was admitted to the neonatal intensive care unit (ICU), a central line was inserted, and the patient was intubated. His blood gases revealed lactic acidosis, hyperkalemia with high anion gap, and normal blood glucose level. Cardiac examination revealed a grade 2 systolic murmur on the base of the heart, with accentuated second heart sound. He had delayed capillary refilling (more than 6 s). His skin was mottled with grayish appearance, and there was no peripheral pulsation in the lower half of his body. The arterial blood pressure in his right arm was undetectable. His arterial blood pressure was 64/30 mmHg, mean pressure 41 mmHg as measured from his left arm, heart rate of 163 bpm. On examination, the patient showed no dysmorphic feature, and he was in poor general condition, feeble crying, with marked tachypnea and tachycardia. The antenatal period was uneventful with no maternal illness or exposures, and the delivery was vaginal and was uneventful too. Sometimes the aortic anatomy is very complex and requires unusual surgical techniques for its repair.Ī full-term, newly born first baby for a non-consanguineous parent, presented 6 days after delivery with severe tachypnea, irritability, poor suckling, poor feeding, and lethargy. It sometimes escapes early diagnosis due to the presence of patent ductus arteriosus and present later with shock and lactic acidosis. ConclusionĪortic interruption is a rare congenital anomaly and is considered an extreme form of aortic coarctation. His aortic anatomy was very complex and he was treated with long extra-anatomical aortic interposition graft. The patient escaped early diagnosis at birth and presented few days later by a picture that mimicked severe sepsis and shock. This case represents a neonate with interrupted aortic arch (type B) and a very long segment of descending aorta hypoplasia and complex anatomy. The diagnosis and surgical treatment of aortic interruption is usually challenging and may require multiple operations throughout the patient’s life. This congenital anomaly rarely occurs as an isolated lesion and is often associated with other intracardiac malformations, most commonly ventricular septal defect and patent ductus arteriosus (PDA). Interrupted aortic arch (IAA) is a congenital malformation of the aortic arch which involves 3 out of 1 million live births.
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