p.54 抽出テキスト

...................................................................................................................................................................... well as X-ray and magnetic resonance imaging fusion, aid in precision assessment.267

4.11.4 Surgical/catheter interventional treatment For follow-up and intervention in patients with Fallot-like repair with transannular patch, see section 4.10; for patients with repair using a valved RVPA conduit, see section 4.14. Patients with pulmonary atresia þ VSD surviving unrepaired to adulthood, or with previous palliative procedures, may actually benefit from modern surgical or interventional procedures.268,269 Patients with good-sized confluent PAs and those with large MAPCAs anatomically suitable for unifocalization, who have not developed severe PVD due to protecting stenosis, should be considered for surgery. Many unrepaired patients may, however, not be suitable for further surgery, mainly because of the complexity of their pulmonary vasculature. It is important to appreciate that while cardiac surgery may improve clinical status or prognosis (the latter is purely speculative), it is also a major cause of mortality. Catheter intervention may include balloon dilation/stenting of collateral vessels to enhance pulmonary blood flow.270 On the other hand, patients with severe haemoptysis may require coiling of ruptured collateral vessels. Survival depends on the complexity of the pulmonary malformations and the results of surgical repair. Survival in palliated patients is significantly lower and has been reported as 60% at 20-year followup. Heart-lung transplantation could possibly be an option for highly selected individuals.

4.11.5 Follow-up recommendations Patients with pulmonary atresia þ VSD should have periodic followup in a specialized ACHD centre (at least once a year). For the management of cyanosis-related multiorgan involvement, see section 3.4.8. Patients with segmental PAH may be considered for targeted PAH therapy; see section 3.4.3.271,272 Symptoms such as dyspnoea, increasing cyanosis, change in the shunt murmur, heart failure, or arrhythmias warrant special attention and should necessitate an earlier review and assessment for intervention.

4.11.6 Additional considerations

• Exercise/sports: those with excellent haemodynamics should be encouraged to exercise regularly, avoiding only extreme isometric exercise. Those with less optimal haemodynamics will be more functionally limited. Extremes of exertion should be avoided, but regular low-intensity physical activity (walking, swimming, even cycling) should be encouraged. • Pregnancy: the risk of pregnancy in repaired patients with good haemodynamics and no history of arrhythmias is low. The risk increases with hypoxaemia, PAH, ventricular dysfunction, heart failure symptoms, and arrhythmias (see section 3.5.7). As microdeletion 22q11 is fairly common with this defect, patients should be checked before pregnancy. • IE prophylaxis: recommended only for high-risk patients (including all unrepaired patients; see section 3.4.6).

4.12 Transposition of the great arteries

4.12.1 Introduction and background TGA is characterized by AV concordance and ventriculo-arterial discordance: the aorta originates from the RV, the PA from the LV. TGA is called simple in the absence of associated congenital anomalies; TGA is called complex in the presence of associated anomalies: VSD (45%), LVOTO (25%), and CoA (5%). Long-term outcome of complex TGA is, regardless of the type of surgical repair, worse than that of simple TGA. The aetiology of TGA is unknown and the pathogenesis is controversial. Familial occurrence exists but is very rare. There is a 2:1 male preponderance. Natural history is extremely poor and survival to adult life without surgical repair is the exception. Surgical techniques have evolved: atrial switch transitioned to arterial switch procedure, and complex TGAs are often operated upon using a Rastelli-type repair. A schematic overview of surgical techniques and their long-term complications in TGA is provided in Figure 9.

4.12.2 Atrial switch operation 4.12.2.1 Clinical presentation after atrial switch Older adults with a simple TGA have a Mustard or Senning atrial switch procedure. The most common complications are: • Systemic RV dysfunction and failure. • Secondary progressive TR (systemic AV valve). • Bradycardia and chronotropic incompetence due to loss of sinus rhythm; AV conduction is usually intact. • Supraventricular tachyarrhythmia, typically cavotricuspid isthmus-dependent flutter, followed by macro-reentry circuit related to surgical incisions/scars; AF may occur at older age. High heart rates are often haemodynamically poorly tolerated because of the inability to increase preload, a consequence of the (restrictive) atrial baffles. Bradycardia due to SND can promote AT. • Ventricular tachyarrhythmias: primary polymorphic VT or VF due to poor ventricular function and heart failure-related mechanism, or monomorphic VT due to scar/incision/patch-related reentry in repaired complex TGA; secondary VT or VF, preceded by supraventricular tachycardia (SVT) with rapid conduction and consecutive myocardial ischaemia due to the very low stroke volume associated with the SVT. • Baffle stenosis, either superior baffle (most common) or inferior baffle obstruction. • Baffle leakage, with either LR shunt giving rise to pulmonary overflow or RL shunting in the presence of distal flow obstruction, with cyanosis or paradoxical embolism. • Pulmonary veins/venous atrial obstruction, most often at the site where the pulmonary veins connect to the pulmonary venous atrium/RA. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026