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CHAPTER SUMMARY AND CENTRAL ILLUSTRATION
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Chapter Summary
This chapter provides an overview of the foremost right-sided lesions affecting adults with congenital heart disease (see Fuster and Hurst’s Central Illustration), focusing on pathophysiology and clinical management. Right-sided cardiac lesions are typically congenital and include a wide spectrum of conditions of differing complexity. Even “simple” right-sided heart lesions can cause significant right ventricular pressure or volume overload, obstruction of flow to the lungs, and systemic venous congestion. More complex lesions, such as Tetralogy of Fallot, also consist of predominantly right-sided anatomic features and long-term residual right-sided lesions may be present after repair (eg, pulmonary regurgitation). Advances in diagnosis and timely surgical intervention have considerably improved the outlook of these lesions and have minimized long-term complications. However, adults with congenital heart disease remain at risk of long-term sequelae, such as exercise intolerance, right-sided heart failure, and arrhythmias. Long-term specialist care is, therefore, essential, even after successful surgical repair. Expert input and individualized advice are also required in special situations, including pregnancy, noncardiac surgery, and when determining the type and intensity of exercise or sports that patients can practice.
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Pulmonary stenosis (PS) comprises of conditions that result in obstruction to flow from the subpulmonary ventricle to the lungs. PS can range in severity, from mild to severe, and in location (subvalvar, valvar, and supravalvar/peripheral PS). Valvar PS occurs in approximately 7% of patients with congenital heart disease (CHD) and is the most common type of right-sided obstructive lesion.
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Early diagnosis and advances in management have meant that isolated valvar PS is currently considered a relatively benign condition that can be treated by surgical or percutaneous means, carrying a good prognosis, even though short- and long-term complications can occur, and lifelong specialist follow-up is advisable.
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PS is associated with genetic disorders, commonly RASopathies (eg, Noonan syndrome), Williams syndrome, and Alagille syndrome, as well as GATA4 mutations.1,2 PS can rarely be rheumatic, due to carcinoid, or may be caused by infective endocarditis. Stenosis of a prosthetic pulmonary valve is not uncommon in adult CHD (ACHD), after pulmonary valve replacement in patients who have undergone a Ross procedure for aortic valve disease, or in patients with pulmonary regurgitation (PR) after repair of PS (open valvotomy or balloon valvuloplasty) or Tetralogy of Fallot (ToF).
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Anatomical Features and Associated Lesions
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Valvar PS (Fig. 67–1) is usually an isolated lesion and typically consists of fusion of the commissures of adjacent cusps of the pulmonary valve, leaving a central opening of varying degrees depending on the severity of the stenosis (Fig. 67–2). The valve may appear dome-shaped and, in adult patients, thickened and calcific. The number ...