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Pulmonary hypertension (PH) is a haemodynamic and pathophysiological disorder associated with a variety of cardiovascular and respiratory conditions, including congenital heart disease (CHD). In this Chapter, we discuss the definition and pathophysiology of pulmonary arterial hypertension (PAH) related to CHD (PAH-CHD) and its evolution, from Victor Eisenmenger’s first report, to its systematic description by Paul Wood. We focus not only on lung histopathology, but also describe current knowledge on cardiac adaptation to PH and the effect of PAH on the large pulmonary arteries. Finally, we introduce the concept of operability of cardiac defects in the presence of PAH and describe current recommendations for identifying PAH-CHD patients who may benefit from repair of their defect, as opposed to those in whom the defect should not be closed.

Autopsy still represents the gold standard procedure to identify the causes of death after transplantation and remains a key investigation in the integration of medical knowledge in cardiac transplantation. In this chapter, we have highlighted the major roles played by this irreplaceable procedure and dissected the many aspects, which should be evaluated during autopsy to reach a correct diagnosis, a full clinicopathological correlation with deep discussion with the clinicians before signing out the final report to ensure that all clinical questions have been addressed during autopsy, its role in the clinical governance of transplant centers for quality control and transplant-related clinical audit, education and research. With the protocol we have given also some practical information for carrying it out correctly and not to miss the unique opportunity for collecting tissues for further studies and setting of bioarchives and biobanking. The assessment of the technical aspects related to the surgical procedure, the identifications of complications related to transplantation and to the immunosuppression and the comorbidities are some of the points which will be addressed in more details.

Hypoplastic left heart syndrome (HLHS) [12] represents a complex congenital defect which is characterized by different entities as

The pulmonary valve atresia/critical valve stenosis is a congenital heart defect which develops during the fetal life and is characterized by a wide spectrum of severity as result of the blood flow remodeling process of the right ventricle outflow. In the atretic valve there is absence of communication between the right ventricle outflow and the size of the pulmonary trunk and the right ventricle can vary considerably. Very often massive hypertrophy of the right parietal wall contribute substantially to the hypoplasia of the ventricular cavity. The tricuspid valve is also affected, been very often diminutive and dysplastic.Three types of pulmonary valve morphology can be identified: type a: pulmonary valve atresia with imperforate valve; type b: critical pulmonary valve stenosis with three dysplastic valve leaflets; type c: critical pulmonary valve stenosis with bicuspid or unicuspid valve.

The pathology of aortic valve diseases can manifest at subvalvular, valvular, or supravalvular level. Fetal supravalvular aortic stenosis is very rare, while subvalvular stenosis is encountered more often in the setting of complex congenital defect. The most common fetal aortic valve diseases are represented by atresia or valvular stenosis. This is often characterized by restricted cusps excursion and post-stenotic dilatation of the ascending aorta. In aortic atresia an imperforate membrane is guarding the aortic severe hypoplastic annulus and ascending aorta. The blood flow and pressure reduction on the left side of the heart during gestation impact on the remodeling cardiac process leading to hypoplastic left heart syndrome.