| Popis: |
Patients with congenitally corrected transposition of great arteries (ccTGA) present with heart failure commonly in the fourth or fifth decade of life. Prevalence of ccTGA is < 0.5%, with dextrocardia reported among 20% of these patients. We present a 52-year old female patient with ccTGA and dextrocardia. In 2008, she was first admitted to our institution because of heart failure and diagnosed with ccTGA combined with dextrocardia. She was treated with heart failure medications for approximately ten years which improved her condition. In the January 2017, despite optimal medication, she developed congestive heart failure and required additional rehospitalization with symptoms of congestion and low cardiac output with elevated NT-pro-BNP levels up to 10752 pg/mL. Her cardiac functional status decreased to NYHA (New York Heart Association) class IV. She was treated with inotropic sup- port and careful volume management. Echocardiography confirmed the presence of ccTGA and dextrocardia with situs solitus, with ejection fraction of dilated systemic right ventricle less than 20%. Patient also had severe tricuspid regurgitation and mild mitral regurgitation, mild pulmonic regurgitation with mild subpulmonic obstruction due to accessory fibrous tissue of basal part of interventricular septum. She was at that time evaluated for heart transplantation and placed on the cardiac trans- plant waiting list. Despite medication her condition deteriorated, so invasive haemo- dynamics measurements were repeated revealing RVP 71/6/19mmHg, PCWP 29mmHg, CI 1, 39 L/min/m2, MPAP 45mmHg, PVR 6, 24WU. Pulmonary hyperten- sion may have induced right heart failure in the transplanted heart, so multidisci- plinary heart transplant team decided for long term unloading with ventricular assist device (VAD) as a bridge to heart transplant candidacy. In September 2017 intraperi- cardial VAD (centrifugal continuous flow, fully magnetically-levitated technology) w positioning the inflow cannula in the systemic ventricle, assessment of an outflow cannula in the lateral right side of ascending aorta, in setting of dextrocardia present in our patient. Post procedural hospital course was complicated by mild subpul- monic ventricle failure managed by inotropic support and pump adjustment. Patient was discharged four weeks after implantation of VAD, taking pharmacological treat- ment of warfarin, acetylsalicylic acid and heart failure medication, currently in NYHA class I-II. Implantation of VAD in systemic ventricle in ccTGA has been described in small groups of patients, and to our knowledge in a single patient with ccTGA com- bined with dextrocardia. Accurate imaging (transthoracic echocardiography with contrast, transoesophagic echocardiography, cardiac MSCT) and multidisciplinary heart team is pivotal in successful implantation of small size intrapericardial VAD in patient with complex anatomy as ccTGA and dextrocardia. |
