ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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Background Several clinical risk factors for death and heart transplantation have been identified in patients with Fontan circulation. It is unknown whether cardiac magnetic resonance (CMR) measurements of ventricular size and function are independently associated with these outcomes and further improve risk stratification. Methods and Results Data on Fontan patients who had a CMR study from 1/2002 to 1/2011 were retrospectively reviewed. The endpoint was time to death or listing for heart transplantation after the CMR study. The median age of the 215 patients was 18.3 years [25th, 75th percentiles: 14, 26] with a median age at Fontan of 3.6 years [2.3, 7.1]. Over a median post-CMR follow-up period of 4.1 years [2.6, 6.2], 24 patients (11%) reached the endpoint: 20 deaths, 3 transplants, and 1 transplant listing. In a multivariable Cox regression model with clinical parameters only, protein losing enteropathy (PLE) was associated with transplant-free survival. A multivariable model including clinical and CMR parameters showed that in addition to PLE, ventricular end-diastolic volume (EDVi) >125 mL/BSA1.3 was associated with transplant-free survival. A likelihood-ratio test comparing the 2 models showed that the addition of EDVi resulted in a significantly improved endpoint prediction (P<0.001) — C-index increased from 0.63 to 0.79. Conclusions CMR-derived ventricular EDVi is an independent predictor of transplant-free survival late after the Fontan operation and adds incremental value over clinical symptoms alone for risk stratification.

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Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed. Of 17 referrals for cardiac transplantation, seven Fontan patients underwent CHLT. All patients who underwent CHLT had either advanced fibrosis or cirrhosis on liver biopsy. There were no perioperative deaths. The most common postoperative morbidity was acute kidney injury. Short-term complications include one episode of acute liver rejection but no cardiac rejection greater than 1R. CHLT is an acceptable therapeutic option for patients with failing Fontan physiology who exhibit concomitant advanced liver fibrosis. However, optimal patient selection is currently undefined, and long-term outcomes are not known.

ACHD-OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments.