p.22 抽出テキスト

................................................ anticoagulation and antiplatelet agents should be considered carefully on a case-by-case basis.

3.4.3.3.4 Shunt repair. Because endothelial shear triggers PH,52 surgical/interventional repair of conditions of increased flow are conceptualized to protect the pulmonary vasculature. The threshold PVR permitting reparative surgical closure of a left-to-right (LR) shunt without right heart failure differs for the different shunt lesions (see sections 4.14.4). However, the decision to close a shunt is made using all the available information and is not solely dependent on the haemodynamics obtained at cardiac catheterization;53,54 the decision should only be taken in expert centres. There is no prospective evidence that a treat-and-repair approach in patients with PAH-CHD confers long-term benefits.44,55 No prospective data are available on the usefulness of vasoreactivity testing, closure test, or lung biopsy for assessment of operability.53,54,5660

3.4.3.3.5 Pulmonary arterial hypertension-directed medical therapy. Advanced therapies benefit patients with Eisenmenger syndrome,51 and likely also other PAH-CHD.61,62 According to the 2015 ESC/ERS Guidelines on PH,44pre-capillary PH (PAH), including Eisenmenger syndrome, is a moderate- to high-risk condition and requires a proactive approach using initial63 or sequential combination treatment,64,65 including parenteral prostacyclins.44 Parenteral prostaglandins work best when started early.66 The presence of a central intravenous catheter for parenteral therapy, however, increases the risk of paradoxical embolism and infection in Eisenmenger patients and those with RL shunt lesions. In this setting, subcutaneous or inhaled forms of administration are therefore generally preferred. Exclusions to this rule are patients with closed or coincidental defects meeting strict criteria for vasodilator responsiveness (mainly a decrease of mean PAP >10 mmHg and below 40 mmHg acutely under nitric oxide inhalation) who may be treated with calcium channel blocker therapy only. However, such patients are extremely rare among adults with PAH-CHD. General vasoreactivity testing is not recommended in PAH-CHD.44 Long-term home oxygen therapy may improve symptoms but has not been shown to augment survival in Eisenmenger patients. The use of supplemental oxygen should be restricted to those cases in which it produces a documented, consistent, and significant increase in arterial oxygen saturation and improves symptoms. Secondary erythrocytosis is beneficial for adequate oxygen transport and delivery, and routine phlebotomy should be avoided; for more details see section 3.4.8. In Eisenmenger patients, the endothelin receptor antagonist (ERA) bosentan has been shown to improve 6MWT and decrease PVR after 16 weeks of treatment in World Health Organization (WHO) functional class III patients.67 Although a beneficial effect of bosentan has been shown on exercise capacity and quality of life in this group of patients, an effect on mortality is less well documented. Long-term follow-up showed sustained improvement in symptoms. Experiences with other ERAs and the phosphodiesterase type 5 (PDE-5) inhibitors, sildenafil and tadalafil, show favourable functional and haemodynamic results in patients with PAH-CHD and Eisenmenger syndrome, with less robust evidence. Experience is limited for latest-generation PAH drugs such as macitentan, selexipag, or riociguat in the setting of CHD.66 While patients with simple repaired lesions have been included in the landmark studies for these substances, and are likely to benefit similarly to idiopathic PAH patients, data are currently limited for Eisenmenger patients. A recent randomized controlled trial investigating efficacy of macitentan to improve 6MWT in Eisenmenger syndrome was neutral.68 Most centres follow a sequential symptomorientated treatment strategy in Eisenmenger syndrome, generally starting with an oral ERA or PDE-5 inhibitor and escalating therapy if symptoms persist or in the case of clinical deterioration. Should an adequate improvement in symptoms not be achieved with oral therapy, parenteral options should be considered proactively.

The effect of PAH therapy in patients with segmental PAH remains a matter of debate. While some series reported promising results, there have been cases where therapies were not tolerated.46 Heart-lung or lung transplantation with heart surgery is an option in special cases not responsive to medical treatment but is limited by surgical complexity and organ availability.

3.4.4 Surgical treatment Cardiac surgery in ACHD patients deserves special attention. Even small operations can carry a high risk due to the dysregulation of the delicate balance in which ACHD patients often thrive. This risk cannot be calculated using conventional surgical risk scores. The adult congenital heart surgery score was derived from the Society of

Recommendations for treatment of pulmonary arterial hypertension associated with congenital heart disease

Recommendations Classa Levelb

It is recommended that patients with CHD and confirmed pre-capillary PHc are counselled against pregnancy. I C Risk assessment is recommended in all patients with PAH-CHD.d I C In low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH, initial oral combination therapy or sequential combination therapy is recommended and highrisk patients should be treated with initial combination therapy including parenteral prostanoids.e 6365 I A In Eisenmenger patients with reduced exercise capacity (6MWT distance <450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered followed by combination therapy if patients fail to improve.6769 IIa B

6MWT = 6-minute walk test; CHD = congenital heart disease; PAH-CHD = pulmonary arterial hypertension associated with congenital heart disease; PH = pulmonary hypertension. aClass of recommendation. bLevel of evidence. cThe risk of pregnancy in patients with pre-capillary PH is very high. It may be less in pregnancies of patients with post-capillary PH, therefore, a right heart catheterization is necessary in all patients with a suspicion of pre-capillary PH to confirm the diagnosis. dFor details see 2015 ESC/ERS Guidelines for diagnosis and treatment of PH.44 eFor details about choice of drugs and the recommended risk-adjusted treatment algorithm see 2015 ESC/ERS Guidelines for diagnosis and treatment of PH.44 Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026