ABSTRACT
BACKGROUND: The initial classic Fontan utilising a direct right atrial appendage to pulmonary artery anastomosis led to numerous complications. Adults with such complications may benefit from conversion to a total cavo-pulmonary connection, the current standard palliation for children with univentricular hearts. METHODS: A single institution, retrospective chart review was conducted for all Fontan conversion procedures performed from July, 1999 through January, 2017. Variables analysed included age, sex, reason for Fontan conversion, age at Fontan conversion, and early mortality or heart transplant within 1 year after Fontan conversion. RESULTS: A total of 41 Fontan conversion patients were identified. Average age at Fontan conversion was 24.5 ± 9.2 years. Dominant left ventricular physiology was present in 37/41 (90.2%) patients. Right-sided heart failure occurred in 39/41 (95.1%) patients and right atrial dilation was present in 33/41 (80.5%) patients. The most common causes for Fontan conversion included atrial arrhythmia in 37/41 (90.2%), NYHA class II HF or greater in 31/41 (75.6%), ventricular dysfunction in 23/41 (56.1%), and cirrhosis or fibrosis in 7/41 (17.1%) patients. Median post-surgical follow-up was 6.2 ± 4.9 years. Survival rates at 30 days, 1 year, and greater than 1-year post-Fontan conversion were 95.1, 92.7, and 87.8%, respectively. Two patients underwent heart transplant: the first within 1 year of Fontan conversion for heart failure and the second at 5.3 years for liver failure. CONCLUSIONS: Fontan conversion should be considered early when atrial arrhythmias become common rather than waiting for severe heart failure to ensue, and Fontan conversion can be accomplished with an acceptable risk profile.
Subject(s)
Fontan Procedure/methods , Postoperative Complications/etiology , Adolescent , Adult , Arrhythmias, Cardiac/etiology , Arrhythmias, Cardiac/mortality , Female , Heart Atria/surgery , Heart Failure/etiology , Heart Failure/mortality , Heart Transplantation , Heart Ventricles/surgery , Humans , Imaging, Three-Dimensional , Liver Failure/etiology , Liver Failure/mortality , Male , Morbidity , Postoperative Complications/mortality , Pulmonary Artery/surgery , Retrospective Studies , Survival Rate , Tomography, X-Ray Computed , Treatment Failure , Ventricular Dysfunction/etiology , Ventricular Dysfunction/mortality , Young AdultABSTRACT
Adults with congenital heart disease represent a growing population with challenging and complex medical management. Pulmonary scintigraphy can play a valuable role in the evaluation and care of this patient population. We present a review of the variety of clinical scenarios where pulmonary scintigraphy can be helpful in the evaluation of adults with congenital heart disease, along with technical considerations associated with these studies.
Subject(s)
Heart Defects, Congenital/diagnostic imaging , Lung/diagnostic imaging , Radionuclide Imaging , Adult , Heart Defects, Congenital/complications , HumansABSTRACT
BACKGROUND: The Ross aortic valve replacement (AVR) has been the AVR of choice for children at our center since 1993. Absence or inadequate quality of the pulmonary valve or, less commonly, family or surgeon preference caused us to select an alternative AVR prosthesis for some children. This review compares the outcomes of 42 children who received a non-Ross AVR with 115 children undergoing Ross root replacement at our institution during the most recent 22 years. METHODS: A retrospective chart review of the 42 pediatric non-Ross AVRs was compared with 115 Ross AVRs. The mean age at AVR was 11.0 ± 6.5 years (range, 1 month to 18 years) for the non-Ross and 11.4 ± 5.5 years (range, 6 weeks to 18 years) for the Ross groups. Follow-up was 7.8 ± 6.2 years for the Ross group and 8.7 ± 6.5 years for the non-Ross group. The Ross AVR technique was modified in 2000; these modifications were applied to 72 patients (63%) of the total Ross AVR group. RESULTS: All-cause early and late mortality of the non-Ross AVR group was 17% compared with 4% for the Ross cohort (p = 0.017). The actuarial survival rate at 20 years for the non-Ross AVR group was 81% compared with 94% for the Ross group (p = 0.018). Reintervention rate was 29% (12/38) in the non-Ross group and 28% (32/115) in the entire Ross group (60% [26/43] before 2000, and 8% [6/72] after 2000). Thromboemboli, bleeding, and endocarditis occurred in 2.4%, 1.4%, and 10% in the non-Ross group compared with 0.9%, 2.6%, and 1.7% in the Ross AVR group (p = 0.46, 0.01, and 0.02), respectively. Patients in the non-Ross group had a significantly higher recurrent or persistent aortic valve gradient (>20 mm Hg) than did patients in the Ross group at most recent follow-up (p < 0.05). CONCLUSIONS: Lower mortality, valve-related complications, and better hemodynamics were seen after the Ross AVR than with other types of AVR prostheses. The Ross AVR remains the treatment of choice for children who have an adequate pulmonary valve. Reoperation for Ross root dilatation, regurgitation, or both (only 6% since our modifications in 2000) has markedly reduced the main drawbacks of this AVR technique. The Ross AVR affords the growing child the advantages of a growing, autologous, viable valve substitute and eliminates the lifelong disadvantages of mechanical and xenograft valves.
