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............................................................................................................................................................................. 4.4.3 Diagnostic work-up See section 3.3 for general principles.

Specific clinical findings include a continuous murmur that disappears with development of Eisenmenger syndrome (for differential cyanosis, see section 4.4.2; oxygen saturation should be measured at the upper and lower extremities). • Echocardiography is the key diagnostic technique and provides the diagnosis (may be difficult in patients with Eisenmenger physiology), the degree of LV volume overload, PAP, PA size, and right heart changes. • CMR is indicated when additional quantification of LV volumes and quantification of shunt (Qp:Qs) is needed. • CMR/CCT can further evaluate the anatomy where required. • Cardiac catheterization is required in the case of non-invasive signs of PAP elevation (calculated systolic PAP >40 mmHg or indirect signs when PAP cannot be estimated) to determine PVR. Measurementofpulmonarybloodflowischallenginginthissetting.MeasurementofoxygensaturationinbothleftandrightPAsismandatory. • Exercise testing should be performed in patients with PAH to exclude desaturation of lower limbs.

4.4.4 Surgical/catheter interventional treatment Indications for intervention are summarized in the Recommendation for intervention in patent ductus arteriosus table and in Figure 4. In adults, calcification of the PDA may cause a problem for surgical closure. Device closure is the method of choice, even if cardiac operations are indicated due to other concomitant cardiac lesions, and can be successfully performed in the vast majority of adults with a very low complication rate.168170 Surgery is reserved for the rare patient with a duct too large for device closure or with unsuitable anatomy such as aneurysm formation.

4.4.5 Follow-up recommendations Echocardiographic evaluation should include LV size and function, PAP, residual shunt, and associated lesions. Patients with no residual shunt, normal LV, and normal PAP do not require regular follow-up after 6 months. Patients with LV dysfunction and patients with residual PAH should be followed at intervals of 13 years, depending on severity, including evaluation in specialized ACHD centres.

4.4.6 Additional considerations

• Exercise/sports: no restrictions in asymptomatic patients before or after intervention without PH; limitation to low-intensity sports in PAH patients. • Pregnancy: no significantly increased risk for patients without PH. Patients with pre-capillary PH should be counselled against pregnancy (see section 3.5.7). • IE prophylaxis: limited to high-risk patients (see section 3.4.6).

4.5 Left ventricular outflow tract

obstruction 4.5.1 Valvular aortic stenosis 4.5.1.1 Introduction and background The most common cause for congenital valvular aortic stenosis (AS) is BAV. Up to 80% of patients with a BAV will develop ascending aortic dilatation, which is discussed in section 4.7.2. For the management of AR associated with BAV, see the 2017 ESC/EACTS Guidelines on the management of valvular heart disease.25

4.5.1.2 Clinical presentation and natural history Patients frequently remain asymptomatic for many years. Progression of stenosis varies and depends on initial severity, degree of calcification, age, and atherosclerotic risk factors. In BAV, progression is faster in those patients with greater closure-line eccentricity and an anteroposterior-oriented line of closure. Prognosis is good and sudden death is rare in asymptomatic patients with good exercise tolerance, even when stenosis is severe.171 Once symptoms (angina pectoris, dyspnoea, or syncope) occur, the prognosis deteriorates rapidly. In patients with BAV, cardiac mortality has been reported to be 0.3% per patient-year of follow-up, the frequency of aortic dissection 0.03%, and endocarditis 0.3%. Dilated aortic sinuses and/or ascending aorta have been found in 45% of patients after 9 years of follow-up.172

4.5.1.3 Diagnostic work-up See section 3.3 for general principles. Diagnostic criteria for degree of AS are summarized in Table 12.

Recommendations for intervention in patent ductus arteriosus

Recommendations Classa Levelb In patients with evidence of LV volume overloadc and no PAH (no non-invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs), PDA closure is recommended regardless of symptoms. I C Device closure is recommended as the method of choice when technically suitable. I C In patients who have developed PAH with PVR 35 WU, PDA closure should be considered when there is still significant LR shunt (Qp:Qs >1.5). IIa C In patients who have developed PAH with PVR >_5 WU, PDA closure may be considered when there is still significant LR shunt (Qp:Qs >1.5) but careful individual decision in expert centres is required. IIb C PDA closure is not recommended in patients with Eisenmenger physiology and patients with lower limb desaturation on exercise.d III C

LR = left-to-right; LV = left ventricle/ventricular; PAH = pulmonary arterial hypertension; PAP = pulmonary artery pressure; PDA = patent ductus arteriosus; PVR = pulmonary vascular resistance; Qp:Qs = pulmonary to systemic flow ratio; WU = Wood units. aClass of recommendation. bLevel of evidence. cLV enlargement with increased stroke volume. dThere are limited data available for a precise cut-off, but by clinical experience, this would be given by a fall of arterial oxygen saturation <90%. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026