Congenital Diaphragmatic Hernia (Cdh) With Intrathoracic Spleen And Sudden Cardiac Arrest: A Case Report And Literature Review

Background: A case is reported of sudden unexpected death, prior to surgery, in a preterm infant with a left sided CDH with an intrathoracic spleen and pyloric atresia. This combination is relatively rare. We review the mechanisms for sudden death in CDH and postulate that unusual phasic T wave alternation (TWA), a well described ECG finding in the setting of raised intrathoracic pressure may have induced a ventricular arrhythmia. Methods: A structured database literature search was performed using the terms: diaphragmatic hernia, arrhythmia, sudden death, neonate.No language restriction was used. The literature is reviewed with regards 1) to the outcomes of CDH where there is elevation of intrathoracic pressure caused by the presence of abdominal organs in the chest and 2) electrocardiographic changes associated with increased intrathoracic pressure. Results: There were 4 reports in the literature, 1 case with an intrathoracic spleen and 3 cases with CDH and pyloric stenosis.

Case Report: A female infant girl was born at 36 weeks gestation by spontaneous vaginal delivery, birth weight 2445g. Pregnancy and antenatal scans had been normal and there was no past obstetric history of note. The baby was in poor condition with thin meconium and respiratory distress requiring endotracheal intubation. Her general condition deteriorated over the next 24 hours with need of inotropic support, High Frequency Oscillation and Nitric Oxide for worsening respiratory failure and pulmonary hypertension. Initial chest x-ray showed a large gastric bubble in the left hemithorax with rightward displacement of the mediastinum and heart consistent with the diagnosis of CDH. No gas was seen in the distal bowel raising the suspicion of an obstruction. Ultrasound confirmed the presence of the left lobe of the liver with undilated gut and the stomach. The spleen was not detectable in the initial ultrasound examination. Echocardiography confirmed a structurally normal heart with pulmonary hypertension. After 48h of relative stability, she had a sudden profound desaturation with irregular heartbeat then asystole. There was no recorded event or trigger for this deterioration. Despite cardiopulmonary resuscitation with epinephrine, it was not possible to regain cardiac output. A post mortem examination revealed pyloric / duodenal atresia in addition to the CDH and lung hypoplasia. There were no cardiac or genitourinary anomalies.The left lobe of the liver lay within the chest and the spleen was attached to the stomach in the posterior left chest.

Conclusion: We suggest that a number of different factors may have contributed to the sudden loss of cardiac output in our patient, including arrhythmia and additional anatomical anomalies both directly and indirectly. We suggest close electrocardiographic monitoring in patients with intrathoracically placed abdominal organs. Awareness of the potential for arrhythmia may help improve the outcomes for these babies.