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Major advances in the surgical and medical management of congenital heart disease (CHD) in recent decades have meant that an increasing number of children born with CHD survive to adulthood. In the past, lack of early diagnosis and timely repair resulted in a substantial number of patients developing pulmonary vascular disease, especially those with post-tricuspid shunts. Pulmonary arterial hypertension (PAH) related to congenital heart disease (PAH-CHD) is still one the most common types of PAH.

ACHD patients can be challenging to comprehensively manage as they require a multidisciplinary team approach with specialists working in collaboration in many areas. As the number of patients surviving with severe congenital heart disease increases, so does the complexity of the care required and the number of specialist practitioners involved with this patient group. It is necessary to be prepared for what may occur with any intervention with ACHD patients. The medical team needs to be prepared as well as the patient. This chapter aims to address some of the specific areas that need to be prepared in order for adults with congenital heart disease to be cared for at the highest levels and minimizing the morbidities that occur with complex interventions.

The most significant area of growth in adult congenital cardiac practice is in relation to patients with complex univentricular hearts. These patients represent the extreme end of the ACHD spectrum and demonstrate unique manifestations of heart failure. This chapter describes, in detail, the underlying pathophysiology of these changes focusing on patients who have undergone Fontan or cavopulmonary operations. The possible therapeutic options for these patients are explained in terms of their specific impact on the abnormal haemodynamics. The multisystem impact of heart failure and the importance of other end-organ involvement are discussed particularly in the context of timely referral for transplant assessment.

The presence of pulmonary arterial hypertension (PAH) increases morbidity and mortality in patients with congenital heart disease (CHD). PAH associated with CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of pulmonary vasodilators in this population, but, despite recent advances, mortality remains relatively high. Currently, there are limited data to guide the use of pulmonary vasodilators in this population. Furthermore, particular subgroups such as patients with Fontan circulation or “segmental” pulmonary hypertension are challenging to manage and even more challenging to treat. The current chapter reviews the different therapies for PAH in this growing and challenging population.