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Chylothorax following bidirectional Glenn and Kawashima procedure in nine-month infant with interrupted Inferior Vena Cava with azygos continuation

Presenting Author Academic/Professional Position

Jennifer Adams

Academic Level (Author 1)

Medical Student

Discipline/Specialty (Author 1)

Pediatrics

Discipline Track

Clinical Science

Abstract Type

Research/Clinical

Abstract

Chylothorax is a rare but potentially life-threatening complication in children with single-ventricle congenital heart disease palliated with bidirectional cavopulmonary shunt procedure. Patients with anomalous venous anatomy such as interrupted inferior vena cava (IVC) and azygos continuation to the superior vena cava (SVC) are typically palliated with the Kawashima operation. Because the volume of venous blood directed to the pulmonary arteries is substantially greater in the presence of interrupted IVC, the Kawashima operation results in a near Fontan-like circulation. The lymphatic system is adversely affected by Fontan physiology. HLHS patients who develop recurrent chylothorax or pleural effusion may have worse long-term survival outcomes.

Case Presentation

Our patient is a 9-month old male with single ventricular cyanotic heart disease admitted for respiratory distress secondary to chylothorax. His complex medical history includes double outlet right ventricle in newborn, congenital pulmonary valve stenosis, hypoplastic left ventricle, Right Kawashima procedure. On arrival, he suffered from progressive shortness of breath and cyanosis with oxygen saturation of 80% with high-flow nasal cannula. Chest x-rays showed extensive right-sided effusion and progressive worsening of pulmonary opacification. 500 mL of chylous pleural fluid was extracted after chest tube placement and pleural effusion analysis confirmed chylothorax. Internal jugular ultrasound showed bilateral patent internal jugular and subclavian veins. Driscoll postoperative chylothorax management protocol was initiated, beginning with Medium-chain-triglyceride (MCT)-rich milk formula feeds, then IV Octreotide 1 mL/kg/hr for one week. Chest tube was removed on POD 10 when pleural effusion decreased to 5 mL/day.

Conclusion

Chylothorax occurrence in the context of with central venous pressure and pulmonary artery pressures are acceptable for Fontan-like physiology and may suggest underlying lymphatic malformation and warrant further investigative tests. Preoperative assessment of lymphatic perfusion may improve preoperative risk stratification and identify patients in whom lymphatic intervention might reduce the risk of early postoperative cardiac surgery morbidity.

Presentation Type

Poster

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Chylothorax following bidirectional Glenn and Kawashima procedure in nine-month infant with interrupted Inferior Vena Cava with azygos continuation

Chylothorax is a rare but potentially life-threatening complication in children with single-ventricle congenital heart disease palliated with bidirectional cavopulmonary shunt procedure. Patients with anomalous venous anatomy such as interrupted inferior vena cava (IVC) and azygos continuation to the superior vena cava (SVC) are typically palliated with the Kawashima operation. Because the volume of venous blood directed to the pulmonary arteries is substantially greater in the presence of interrupted IVC, the Kawashima operation results in a near Fontan-like circulation. The lymphatic system is adversely affected by Fontan physiology. HLHS patients who develop recurrent chylothorax or pleural effusion may have worse long-term survival outcomes.

Case Presentation

Our patient is a 9-month old male with single ventricular cyanotic heart disease admitted for respiratory distress secondary to chylothorax. His complex medical history includes double outlet right ventricle in newborn, congenital pulmonary valve stenosis, hypoplastic left ventricle, Right Kawashima procedure. On arrival, he suffered from progressive shortness of breath and cyanosis with oxygen saturation of 80% with high-flow nasal cannula. Chest x-rays showed extensive right-sided effusion and progressive worsening of pulmonary opacification. 500 mL of chylous pleural fluid was extracted after chest tube placement and pleural effusion analysis confirmed chylothorax. Internal jugular ultrasound showed bilateral patent internal jugular and subclavian veins. Driscoll postoperative chylothorax management protocol was initiated, beginning with Medium-chain-triglyceride (MCT)-rich milk formula feeds, then IV Octreotide 1 mL/kg/hr for one week. Chest tube was removed on POD 10 when pleural effusion decreased to 5 mL/day.

Conclusion

Chylothorax occurrence in the context of with central venous pressure and pulmonary artery pressures are acceptable for Fontan-like physiology and may suggest underlying lymphatic malformation and warrant further investigative tests. Preoperative assessment of lymphatic perfusion may improve preoperative risk stratification and identify patients in whom lymphatic intervention might reduce the risk of early postoperative cardiac surgery morbidity.