International journal of cardiology. Congenital heart disease最新文献

Background

Cardiopulmonary exercise testing (CPET) is used in evaluation of repaired tetralogy of Fallot (rTOF), particularly for pulmonary valve replacement need. Oxygen pulse (O₂P) is the CPET surrogate for stroke volume and peripheral oxygen extraction.

Objectives

This study assessed O₂P curve properties against non-invasive cardiac output monitoring (NICOM) and clinical testing.

Methods

This cross-sectional study included 44 rTOF patients and 10 controls. Three new evaluations for O₂P curve analysis during CPET were developed. Best fit early and late regression slopes of the O₂P curve were used to calculate: 1) the early to late ratio, or “O₂ pulse response ratio” (O₂PRR); 2) the portion of exercise until slope inflection, or “flattening fraction” (FF); 3) the area under the O₂P response curve, or “O₂P curve area”.

Results

rTOF patients (median age 35.2 (27.6–39.4); 61% female) had a lower VO₂ max (23.4 vs 45.6 ml/kg/min; p < 0.001) and O₂P max (11.5 vs 19.1 ml/beat; p < 0.001) compared to controls. Those with a FF occurring <50% through exercise had a lower peak cardiac index and stroke volume, but not VO₂ max, compared to those >50%. FF and O₂P curve area significantly correlated with peak cardiac index, stroke volume, left and right ventricular ejection fraction, and right ventricular systolic pressure.

Conclusion

CPET remains an integral part in the evaluation of rTOF. We introduce three non-invasive methods to assess exercise hemodynamics using the O₂P curve data. These evaluations demonstrated significant correlations with stroke volume, cardiac output, and right ventricular pressure.

Background

Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia.

Methods

We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code – Short List. Methodological limitations were carefully documented.

Results

From 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %).

Conclusion

The data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.

Chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) occurs when thromboemboli in pulmonary arteries fail to resolve completely. Pulmonary artery obstructions due to chronic thrombi and secondary microvasculopathy can increase pulmonary arterial pressure and resistance leading to chronic thromboembolic PH (CTEPH). Mechanical interventions and/or PH medications can improve cardiopulmonary haemodynamic, alleviate symptoms, and decrease mortality risk. Imaging is pivotal throughout the CTEPD management journey, spanning diagnosis, treatment planning, and assessing treatment outcome. With just computed tomography (CT) pulmonary angiogram and right heart catheterisation, an experienced multidisciplinary team can determine surgical candidacy in most cases. Dual energy CT, lung subtraction iodine mapping CT, and dynamic contrast-enhanced magnetic resonance imaging (MRI) offer comparable sensitivities with ventilation-perfusion scintigraphy in diagnosing CTEPD. Pulmonary angiogram with digital subtraction angiography although considered the gold standard for assessing thrombi extent and vasculature morphology is now mostly used to assess targets for balloon pulmonary angioplasty. Advancements in CT modalities and innovative MRI metrics offer better insight into CTEPD management but are limited by the availability of technology and expertise. Learning from current artificial intelligence application in medical imaging, there is promise in tapping the wealth of data provided by CTEPD imaging through automating cardiopulmonary and vascular morphology analysis.

Pulmonary hypertension (PH) is a serious potential complication of some congenital heart diseases (CHDs). PH encompasses a range of diseases which may be idiopathic or inherited, or secondary to cardiac, respiratory, systemic or thromboembolic conditions, amongst others. Our increasing understanding of the normal ranges of pulmonary haemodynamics, as well as evidence supporting the benefits of early treatment, has resulted in a number of recent revisions to the haemodynamic definition of PH. In this Review Article, we report on the recent updates to haemodynamic definitions and classification of PH, as reflected in the 2022 Pulmonary Hypertension Guidelines and particularly focus on the CHD related sub-type of PH, where the aetiology is often multi-factorial.

Background

Timely diagnosis of heart failure (HF) in patients with a systemic right ventricle (sRV) is difficult but important since clinical deterioration is fast once HF develops. We aimed to compare echocardiography and biomarker profile between sRV patients with and without HF and patients with a systemic left ventricle diagnosed with HF (sLV-HF).

Methods and results

Eighty-seven sRV patients and 30 sLV-HF patients underwent echocardiographic evaluation and blood sampling. Compared to sRV patients without HF, sRV-HF patients had more remodeling of the subpulmonary LV (spLV) (internal diameter 3.9 cm [3.3–5.7] vs 3.4 cm [2.9–3.9], P = 0.03, posterior wall 0.93 cm [0.76–1.20] vs 0.71 cm [0.59–0.91], P = 0.006) and lower spLV systolic function: ejection fraction (59 % ± 14 vs 70 % ± 10, P = 0.011), mitral annular plane systolic excursion (1.7 cm ± 0.5 vs 2.1 cm ± 0.4, P = 0.003), fractional area change (47 % [38–58] vs 59 % [51–70], P = 0.002) and lateral strain rate (−1.2/s ± 0.46 vs −1.5/s ± 0.39, P = 0.016). Inflammatory biomarkers were higher in sRV-HF patients compared to those without HF: red cell distribution width (13.3 fL [12.8–14.1] vs 12.6 fL [12.3–13.1], P < 0.001), neutrophil lymphocyte ratio (NLR, 3.7 [2.2–4.9] vs 2.4 [1.9–3.0], P = 0.015), C-reactive protein (CRP, 2.5 mg/dL [1.0–4.2] vs 1.2 mg/dL [0.0–2.0], P = 0.005) and compared to sLV-HF patients (NLR (3.7 [2.2–4.9] vs 2.5 [1.7–3.3], P = 0.044) and CRP (2.5 mg/dL [1.0–4.2] vs 0.85 mg/dL [0.6–2.0], P = 0.006).

Conclusion

Biventricular echocardiographic evaluation with a focus on the subpulmonary LV together with assessing inflammatory status in sRV patients could help in an earlier detection of HF.

Background

The Norwood operation (NO) for infants with univentricular physiology has high interstage mortality. This study evaluated outcomes and risk factors for mortality following NO.

Methods

Retrospective single-center study of patients undergoing NO from 2010 to 2020. Analysis used appropriate statistics.

Results

Of 269 patients undergoing NO, 213 (79.2 %) survived to discharge. Non-survivors had longer bypass times, delayed sternal closure, required nitric oxide, higher vasoactive scores, required post-operative catheterization, Extracorporeal Life Support (ECLS), and longer ventilation (p < 0.05). Logistic regression showed moderate-severe atrioventricular valve regurgitation on intraoperative TEE (OR 2.6), requiring nitric oxide (OR 2.63), delayed sternal closure (OR 2.94), post-operative catheterization (OR 10.48), and ECLS (OR 14.54) increased mortality odds (p < 0.05). Multivariable analysis confirmed catheterization (aOR 10.48) and ECLS (aOR 14.54) as significant predictors. Of survivors, 26 (12.3 %) developed new morbidity, 9 (4.2 %) had unfavorable outcomes. Functional status improved from 6.0 to 8.04, mainly in feeding and respiratory domains (p < 0.0001).

Conclusions

Norwood survival was 79.2 %. Requiring post-operative catheterization and ECLS significantly increased mortality risk. Multicenter evaluation of these modifiable risk factors is needed to improve outcomes in this high-risk population.