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diminished exercise capacity will deteriorate further. Eventually, overt heart failure may develop, in addition to the cyanosis. In rare cases, with a well-balanced haemodynamic situation, ventricular dysfunction does not develop, and survival until the fifth, sixth, and even seventh decade has been reported.

4.15.3 Diagnostic work-up See section 3.3 for general principles. Clinical findings include central cyanosis, clubbing of fingers and toes, and often an asymmetric chest with a precordial heave at the side where the heart lies in the chest. Scoliosis is a common problem. The second heart sound is typically single, but the rest of the auscultation depends on the associated abnormalities. ECG may reveal rhythm or conduction disturbances. Atrial reentrant tachycardia with 2:1 block and only modest tachycardia may be easily overlooked. • TTE is the key diagnostic technique, providing information on anatomy and monitoring of cardiac function during follow-up. The segmental approach is required in the echocardiographic examination; UVHs are always complex and can present with a wide range of abnormalities in situs, orientation, and connections. Fundamental TTE parameters/issues/items in the diagnosis of UVHs are:

- Abdominal and atrial situs. - Position of the heart in the chest and position of the apex. - Veno-atrial, AV, and ventriculo-arterial connections. - Morphological and haemodynamic information has to be obtained on the entire heart. - Exact anatomy of the ventriculo-arterial connection and its functional status has to be assessed, with special focus on obstruction towards the aorta or pulmonary vascular bed. - AV valve function should be evaluated, with special focus on regurgitation. - Ventricular function/hypertrophy. - ASD/VSD type, size, number, location. - Ascending aorta, aortic arch, and descending aorta; detect/ exclude coarctation. - PAs  common trunk, branches, and sources of pulmonary blood supply. - Visualization of shunts (BlalockTaussig, Waterston, etc.). TOE may be indicated in cases of inadequate TTE images. • CMR is the imaging modality of choice for extracardiac anatomy, including veno-atrial and ventriculo-arterial connections (CCT is an alternative). Detailed morphological information of intracardiac anatomy can also be obtained. CMR is also the method of choice for quantification of ventricular volumes, EF, and relative distribution of blood flow in the left and right lungs. • Cardiac catheterization is required when intervention is considered for haemodynamic assessment, in particular PAP and transpulmonary gradient (PVR is often difficult to assess in this setting). It is mandatory when patients are evaluated for a Fontan operation. Evaluation of systemic-to-PA or Glenn shunts  and their sequelae (stenosis of the pulmonary branches) and other vascular anomalies (arteriovenous collateral vessels, fistulas, etc.)  may also require catheterization. Interventional treatment, such as pulmonary valvotomy to increase pulmonary blood flow in cases of severe PS, is debatable.

If the clinical situation is stable, the (frequently high) risk of any type of surgical intervention should be weighed very carefully against the possible benefit. A Fontan operation can only be considered in very well selected patients (see section 4.16). For patients with severe cyanosis, with decreased pulmonary blood flow without elevated PVR, a bidirectional Glenn shunt (SVC-to-PA) can be an option. If a systemic-to-pulmonary shunt (e.g. arteriovenous axillary fistula or systemic artery pulmonary shunt) is the only option (bidirectional Glenn shunt not sufficient or PAP not low enough for this shunt), the benefit of increased pulmonary blood flow should be weighed against increased volume load to the systemic ventricle. For transplantation, previous sternotomies/thoracotomies, aorto-pulmonary collaterals, and the multisystem nature of cyanotic CHD are technical and medical challenges and limit the outcome.

Special considerations and recommendations for intervention in univentricular heart Recommendations Classa Levelb It is recommended that adults with unoperated or palliated UVHs undergo careful evaluation in specialized centres, including multimodality imaging as well as invasive work-up to decide whether they may benefit from surgical or interventional procedures. I C Only well-selected symptomatic cyanotic patients, after careful evaluation [low pulmonary vascular resistances, adequate function of the AV valve(s), preserved ventricular function], should be considered candidates for a Fontan circulation. IIa C Patients with increased pulmonary blood flow  unlikely at adult age  should be considered for PA banding or tightening of a previously placed band. IIa C Patients with severe cyanosis and decreased pulmonary blood flow, but without elevated PVR or PAP, should be considered for a bidirectional Glenn shunt. IIa C Patients with severe cyanosis and decreased pulmonary blood flow not suitable for a Glenn shunt may be considered for a systemic-to-PA shunt. IIb C Heart transplantation and heart-lung transplantation should be considered when there is no conventional surgical option in patients with poor clinical status. IIa C

AV = atrioventricular; PA = pulmonary artery; PAP = pulmonary artery pressure; PVR = pulmonary vascular resistance; UVH = univentricular heart. aClass of recommendation. bLevel of evidence. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026