An 11-year-old boy was admitted to the intensive care unit w | Figure 1
An 11-year-old boy was admitted to the intensive care unit with respiratory distress and expectoration of bronchial casts every few days. He had a history of dextro-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) and coarctation of the aorta, for which he underwent neonatal arch repair, an arterial switch operation and VSD repair. The operations were complicated by a long period of renal failure, from which he recovered.
Subsequently, he underwent five more sternotomies to address the following issues:
- Regurgitation of both semilunar valves
- Subvalvar and supravalvar stenosis
- Recurrent arch obstruction
- Bilateral branch pulmonary artery stenosis
At age 9 years, he required the following additional surgeries:
- Anterior aortoventriculoplasty with implantation of a 21-mm mechanical aortic valve
- Right ventricular muscle bundle resection
- Replacement of the pulmonary valve and proximal branch pulmonary artery branches with a bifurcated pulmonary homograft
Over the prior two years, he developed severe tricuspid regurgitation (Figure 1), elevated right ventricular pressures and severe homograft valve regurgitation with mild stenosis of the branch pulmonary arteries. Despite having no related symptoms, his liver had been 4 cm below the right costal margin. Until this presentation, he had been relatively stable on diuretic therapy.
Physical examination at presentation was particularly remarkable for an even more enlarged liver (5.5 cm below the right costal margin) and pronounced jugular venous distention (Figure 2).
Cardiac catheterization confirmed elevated right-sided diastolic pressures. MR lymphangiogram revealed collateralization of obstructed lymphatic vessels (Figure 3).
The team adopted a staged approach in which the pulmonary artery and pulmonary valve issues were first corrected, as these could be approached percutaneously. The patient underwent left pulmonary artery stenting and transcatheter pulmonary valve replacement. Despite that, he progressed to daily cast formation and respiratory failure.
The decision-making at this point was to proceed to a more invasive strategy to address the remaining right-sided lesions. He underwent surgical repair of the tricuspid valve, with improvement of his right-sided pressures.