The Failing Mustard: Combined Interventional-Surgical Treatment of a Complex Case with PV Baffle Stenosis and Constrictive Pericarditis

Objectives: Adults patients who underwent atrial switch operation represent often a clinical challenge. Information about prior operations and the institutions where the operation was performed is often not accessible but, if at hand, can be helpful in treating complex patients.

Methods: We report on a 40-year-old female patient who presented to our unit with increased shortness of breath, pleural effusions, and massive ascites (1–2 L/d). The patient suffered from d-TGA and VSD. She underwent a Mustard procedure with VSD closure 1988 in former East Germany (GDR). Because of baffle stenosis and leakage, as well as sick sinus syndrome, she underwent a re-Mustard operation and implantation of an epicardial pacemaker in 1995.

Result: Echocardiography limited due to a poor sonographic window, showed a moderate decrease of the systemic right ventricular function with signs of pulmonary hypertension. Catheterization showed a predominantly postcapillary pulmonary hypertension (90% of systemic) with a wedge pressure of 28 and an end-diastolic pressures of the right ventricle of 16mmHg, revealing a severe stenosis of the pulmonary venous (PV) baffle. Systemic venous pressure was also elevated (24 mm Hg). A CP-stent was retrogradely implanted in the stenotic PV baffle and subsequently dilated to 18 mm with a high-pressure balloon. The effusions persisted, therefore the baffle stent was redilated with a 20 mm high-pressure balloon with almost no residual gradient. Systemic venous pressure was still elevated with 25 mm Hg. Pulmonary hypertension decreased to two-thirds of the systemic pressure. Pleura effusions disappeared, but after a transient decrease ascites persisted like before. A follow-up CT scan showed very dense and thick pericardium (especially anterior) and the surgeon remarked that in the past some institutions inserted an artificial membrane between sternum and heart. We postulated that this could explain the diastolic dysfunction of our patient. The patient underwent surgery which revealed a closed pericardium with a calcified artificial membrane, which was removed. Effusions terminated within 2 days and the patient remains free of ascites over a 6-month follow-up. Clinically she improved in the following weeks with a better threshold for physical activity.

Conclusion: GUCH patients can be very complex, knowing the history of performed surgical techniques can be extreme helpful.

No conflict of interest has been declared by the author(s).