Eric V. Krieger

Background: Cardiac resynchronisation therapy (CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV. Methods: This is an international, retrospective study including patients >18 years from 33 centres with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA. The primary endpoint included overall survival and survival free from HF. The secondary endpoint was a composite of death, hospitalisation for HF, heart transplant, mechanical support and ventricular tachycardia/implantable cardioverter-defibrillator therapies. Results: We identified 105 out of 1721 patients (3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 (1.6–8) years. QRS improvement was limited to those with previous pacing (167±35 vs 154±28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peak VO ₂ and tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though they were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, anatomy, presence of complex disease, previous HF and sRV dysfunction at baseline. At univariable analysis, CRT (HR 4.39–95%, CI 1.6 to 11.9; p=0.003), older age and moderate-to-severe sRV dysfunction at baseline were predictive of death, while CRT (HR 3–95%, CI 1.3 to 7; p=0.01) and sRV dysfunction were associated with HF admission. By multivariable analysis, CRT (HR 8.8–95%, CI 2.9 to 26.6; p=0.0001) and age (HR 1.1%–95%, CI 1.01 to 1.15; p<0.0001) were independently associated with poorer outcome. Conclusion: In this retrospective study in the largest population thus far described with an sRV, CRT implant was not associated with improved survival, even after controlling for key confounders.

Background: Cardiac Resynchronization Therapy(CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV. Methods: This analysis was part of an international, retrospective study of data from 33 centers including patients >18years with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA (ccTGA). The primary endpoint was overall survival. The secondary endpoint was a composite of death, hospitalization for HF, heart transplant, mechanical support, ventricular tachycardia/ICD therapies. Results: We identified 105 out of 1,721 patients(3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 [1.6-8] years. There was no overall QRS improvement (157±37 Vs 153±31 ms; p=0.2), which was limited to those with previous pacing (167±35 Vs 154± 28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peakVO ₂ , tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though there were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, morphology (ccTGA/TGA), presence of complex disease, previous HF admission and sRV dysfunction at baseline. At univariable analysis, CRT (OR 4.02-95% CI:1.48-10.89; p=0.006), older age, and moderate to severe sRV dysfunction at baseline were predictive of death. By multivariable analysis, CRT (OR 2.1-95% CI:1.2-3.8; p=0.008), age (OR 1.03-95% CI:1.01-1.06; p=0.001) and moderate to severe sRV dysfunction (OR 2.5-95% CI:1.3-4.4; p=0.002) were independently associated with poorer outcome. After matching using only patients with subpulmonary ventricle pacing as controls, overall survival was still worse in CRT group (p=0.0057). Conclusion: In this retrospective study in the largest population thus far described with a sRV, CRT implant was not associated to improved survival, even after controlling for key confounders. Further studies with randomization are required to improve the selection of candidates with a sRV most likely to derive benefit from CRT, elucidate the optimal timing, and assess alternative strategies.

BACKGROUND: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle. METHODS: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index 2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support. RESULTS: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5–15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index P P <0.001). CONCLUSIONS: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle.

Postgraduate training programmes allow matriculation in formalised ACHD fellowships from multiple training pathways. In the UK, 2-year ACHD subspecialty training blocks are offered to physicians with a national training number in adult or paediatric cardiology, supplemented by locally arranged, informal fellowship posts. A shorter period of ACHD training can be combined with another subspecialty, such as advanced imaging, for those aiming to work as a cardiologist with …