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............................................................................................................................................................................. 4.1.7 Additional considerations • Exercise/sports: no restrictions in asymptomatic patients before or after intervention without PH, significant arrhythmias, or RV dysfunction; limitation to low-intensity recreational sports in PAH patients (see section 3.5.5). • Pregnancy: low risk in patients without PH, although there may be an increased risk of paradoxical embolism. Patients with pre-capillary PH should be counselled against pregnancy (see section 3.5.7). • IE prophylaxis: recommended for 6 months after device closure (see section 3.4.6). 4.2 Ventricular septal defect 4.2.1 Introduction and background VSD is mostly diagnosed and when indicated treated before adulthood. Spontaneous closure is frequent in childhood. Several locations of the defect within the interventricular septum are possible, and these can be divided into four groups according to their location within the RV (nomenclature varies and synonyms are added):157 • Perimembranous/paramembranous/subaortic/conoventricular (most common, 80% of VSDs; located in the membranous septum with possible extension into inlet, trabecular, or outlet septum; adjacent to tricuspid and aortic valve; so-called aneurysms of the membranous septum i.e. tissue from the septal leaflet of the TV are frequent and may result in partial or complete closure). • Muscular/trabecular (up to 1520%; completely surrounded by muscle; various locations; frequently multiple; spontaneous closure particularly frequent). • Outlet (with or without malalignment of the outlet septum)/ supracristal/subarterial/subpulmonary/infundibular/conal/doubly committed juxta-arterial [5%; located beneath the semilunar valves in the conal or outlet septum; may be associated with progressive aortic regurgitation (AR) due to prolapse of the right aortic cusp and aneurysm of the sinus of Valsalva]. • Inlet/AV canal/AVSD type (inlet of the ventricular septum immediately inferior to the AV valve apparatus; associated with a common AV valve; may be associated with AV septal malalignment and straddling TV; typically occurring in Down syndrome). Often there is one single defect, but multiple defects do occur. VSD is also a common component of complex anomalies, such as TOF, transposition of the great arteries (TGA), and congenitally corrected TGA (ccTGA). Spontaneous closure occurs mainly during childhood and is uncommon in outlet defects.158 The direction and magnitude of the shunt are determined by PVR and systemic vascular resistance, the size of the defect, LV/RV systolic and diastolic function, and the presence of right ventricular outflow tract (RVOT) obstruction (RVOTO) and left ventricular outflow tract (LVOT) obstruction (LVOTO). 4.2.2 Clinical presentation and natural history The usual clinical presentations in adults include: • VSD operated on in childhood, without residual VSD and no PH. • VSD operated on in childhood, with residual VSD. The residual shunt size determines the degree of LV volume overload and the development of PH. • Small VSD with insignificant LR shunt, without LV volume overload or PH (restrictive VSD), which was not considered for surgery in childhood. • VSD with LR shunt, PH (various degrees), and various degrees of LV volume overload (rare). • VSD with RL shunt (Eisenmenger syndrome): large, nonrestrictive, VSD with originally large LR shunt and development of severe PVD eventually resulting in shunt reversal (cyanosis; see sections 3.4.3 and 3.4.8). A large majority of patients with a VSD that has been closed entirely in childhood (spontaneously or surgically), or patients with a small VSD who were either never operated on or who had a residual defect after surgical repair with no LV volume overload on echocardiography, usually remain asymptomatic and do not require further surgery.159 However, an unknown percentage of patients with a small residual VSD develop problems later in life.160 Survival 40 years after closure appears to remain slightly lower than in the general population.161 Several possible problems may occur with advancing age: • A double-chambered RV (DCRV) can develop over time, mostly in perimembranous defects, and may be a result of the jet lesion of the RV endothelium caused by the high-velocity VSD jet. • In the case of an outlet (supracristal) VSD (less commonly perimembranous), there is a risk for prolapse of the right coronary (or non-coronary) cusp of the aortic valve, resulting in progressive AR and formation of a sinus of Valsalva aneurysm. • Arrhythmias can occur, but are less frequent than in other forms of CHD162. • Complete heart block rare nowadays was not uncommon in the earlier years of cardiac smurgery, so can occur, especially in older patients. These patients usually require lifelong pacing. • Late LV dysfunction and heart failure. • Endocarditis. 4.2.3 Diagnostic work-up See section 3.3 for general principles. Specific clinical findings include a holosystolic murmur over the third to fourth left intercostal space, and a precordial thrill may be felt. • Echocardiography is the key diagnostic technique, in general providing the diagnosis and assessment of disease severity. Key findings to provide are location, number, and size of defects, severity of LV volume overload, and estimated PAP. AR due to prolapse of the right or non-coronary cusp must be checked for, especially in the case of outlet (supracristal) and high perimembranous VSDs. DCRV and sinus of Valsalva aneurysm must be excluded. • CMR can serve as an alternative if echocardiography is insufficient, particularly for assessment of LV volume overload and shunt quantification. • Cardiac catheterization is required in case of non-invasive signs of PAP elevation (calculated systolic PAP >40 mmHg or indirect signs when PAP cannot be estimated) to determine PVR. • Exercise testing should be performed in patients with PAH to exclude desaturation. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026