Cardiology in the Young最新文献

Background: Ventricular septal defect is the most common CHD and is complicated by pulmonary hypertension in about 5% of cases. Although long-term outcomes after repair are generally good, persistent pulmonary hypertension has been reported, especially before the widespread treat-and-repair strategy.

Methods: We retrospectively analysed patients aged ≥13 years who underwent repair for ventricular septal defect with pulmonary hypertension at our centre (1970-2024). Pre- and post-operative hemodynamics were assessed. Patients were grouped by preoperative pulmonary vascular resistance (≥3 vs. <3 Wood units). Residual pulmonary hypertension was evaluated by echocardiography.

Results: We studied 115 patients (43 male, 72 female). Follow-up reached 47 years (median 18.5). Residual pulmonary hypertension occurred in 3/115 (2.6%). Median age at intracardiac repair was 5 months. No significant differences were seen between patients with and without residual pulmonary hypertension in age at surgery, preoperative estimated right ventricular pressure, mean pulmonary artery pressure, or postoperative estimated right ventricular pressure. Mean pulmonary artery pressure was higher in the pulmonary vascular resistance ≥3 group than in the <3 group (47 ± 13 vs. 38 ± 11 mmHg, p = 0.019), while postoperative estimated right ventricular pressure did not differ. Among the 3 with residual pulmonary hypertension, 2 underwent intracardiac repair after 1 year of age.

Conclusions: Most patients with ventricular septal defect and pulmonary hypertension had resolution after intracardiac repair; however, residual pulmonary hypertension occurred in about 2.6% during long-term follow-up. Preoperative hemodynamics did not predict persistence. Long-term periodic follow-up remains important after surgery.

Background: Beta blocker non-adherence is a key factor in precipitating cardiac events in patients affected by congenital long QT syndrome. Adherence has been examined in adults affected by congenital long QT syndrome but not in a large paediatric group.

Method: Patients with congenital long QT syndrome types 1 and 2 were identified using a database curated by the Inherited Cardiac Conditions team in Northern Ireland. Medication adherence was reviewed by contacting the patient's GP. A medication possession ratio was then calculated for the year. Adequate adherence was defined as a ratio of ≥0.8, and ideal adherence was defined as a ratio of ≥1.0. Risk factor analysis for poor adherence was performed using multivariable binary logistic regression.

Results: 99 patients' data was suitable for analysis, 71 had LQT1 (78%) and 28 had LQT2 (28%). The median age of the children involved was ten years old. Over 36,135 days the median medication possession ratio of this patient group was 0.92. 56 patients (57%) had at least adequate adherence, of these 44 patients (44%) had ideal adherence. In contrast 43 patients (43%) had less than adequate adherence and of these six patients (6%) were completely non-adherent. Increased deprivation was significantly associated with "less than" ideal adherence Odds Ratio (OR) 1.2 95% confidence intervals CI (1.1-1.4).

Conclusion: Adherence in the paediatric cohort was mostly in the "adequate range." Increased deprivation is a risk factor for "less than" ideal adherence. A small minority of patients can be identified as completely non-adherent by checking prescription records. Future studies should focus on elucidating barriers and enablers to ideal adherence in this population.

Interrupted aortic arch is a rare congenital heart anomaly characterised by a complete discontinuity between the ascending and descending aorta, accounting for approximately 1-1.5% of CHDs. Its incidence in live births ranges from 3 to 20 per million. Survival into adulthood is only possible with the presence of a well-developed collateral circulation. In this case, a 49-year-old male patient presented with acute anterior myocardial infarction, and during emergency coronary angiography, discontinuity of the aortic arch was noticed. The procedure was successfully completed via radial access after femoral access failed. Subsequent thoracic computed tomography angiography confirmed the diagnosis of Type A interrupted aortic arch and revealed widespread collateral arterial circulation. No blood pressure difference was detected between the right-left arms and lower extremities, and the patient remained asymptomatic. This case demonstrates that this rare anomaly in adults can be incidentally detected during emergency procedures and that radial access is an effective alternative in overcoming anatomical obstacles.

We report a rare case of coronary sinus ostium atresia diagnosed due to impaired cardiac function caused by Marshall vein stenosis, which occurred during surgical treatment. Transcatheter perforation of the coronary sinus ostium and stenting of the Marshall vein stenosis improved ventricular function, enabling subsequent surgical enlargement. This staged strategy minimised surgical risks and demonstrated a reliable and effective treatment for such challenging conditions.

Congenital extrahepatic portosystemic shunt (CEPS) is an uncommon condition in which the portal vascular system drains into the systemic veins. We describe an unusual case of CEPS with congenital heart disease, presenting with aneurysmal enlargement of the pulmonary artery and symptoms of airway compression, highlighting the importance of evaluating for additional pathology in case of unexplained oxygenation defect or pulmonary hypertension.

Conclusion: This case illustrates a rare presentation of Wilson's disease, as ventricular arrhythmia-induced syncope was the initial and only manifestation in a young child before the development of classic hepatic or neurological signs. It underscores the importance of considering metabolic and genetic aetiologies in paediatric arrhythmias, especially when accompanied by abnormal liver function. Moreover, early recognition and initiation of chelation therapy can prevent disease progression and enable timely screening and management of affected family members.

Background: Single ventricle pulmonary arteriovenous malformations are poorly understood and variably assessed in published literature. To improve our understanding of single ventricle pulmonary arteriovenous malformations and facilitate multi-centre studies, it will be necessary to have uniform clinical practice patterns among paediatric heart institutions.

Objectives: The aim of this study was to assess paediatric interventional cardiologists' clinical perspectives and practice patterns for diagnosing single ventricle pulmonary arteriovenous malformations.

Methods: We surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap.

Results: Among 253 Congenital Cardiovascular Interventional Consortium members, a total of 55 (21.7%) paediatric cardiology interventional attending physicians completed the survey. There was near unanimity (98%) that pulmonary arteriovenous malformations develop due to lack of hepatic vein blood flow to the lungs; however, there was wide variation among practice patterns. A minority (20%) of respondents perform bubble contrast echocardiograms (bubble studies) more than half the time pre-Fontan, whereas many (31%) almost never (< 5% of cases) perform bubble studies pre-Fontan. Most respondents reported that they did not perform bubble studies because results do not impact clinical decision making pre-Fontan (56%) or post-Fontan (60%). Many respondents (49%) do not have a typical volume of agitated saline that they inject for bubble studies.

Conclusions: Clinical practice patterns vary widely among paediatric cardiology interventionalists. A standardised clinical approach, new diagnostic tools, or both are needed to standardise our field's approach to diagnosing, studying, and treating single ventricle pulmonary arteriovenous malformations.

Purpose: The impact of arch anatomy on the prognosis of aortic coarctation of the aorta (COA) is not well established. We aim to assess the relationship between arch anatomy and the short- and long-term prognosis after surgical repair.

Methods: Patients with COA operated on the period between 11/2007 and 03/2016 were retrospectively recruited. Anatomical analysis of the aortic arch was done using Multidetector CT with measurement of the inter-branch distances between left common carotid artery, innominate artery, and left subclavian. We classified patients into group I, whose distance ratio (LCCA- IA)/(LSCA-IA) is short and less than 50%, and group II with such ratio ≥ 50%.

Results: Seventy-three patients were recruited. The distance (LCCA-IA) had a range of Zero (common origin) to 22.3 mm. The distance ratio (LCCA-IA)/(LSCA-IA) ranged between Zero and 89%. Group I had a significantly higher incidence of adverse outcomes, including recoarctation, re-elevation of blood pressure, and re-elevation of pressure gradient, compared to Group II (p = 0.0001, 0.011, and 0.014, respectively). A positive correlation exists between the distance ratio and the residual SPG across the repaired segment (P = 0.0001). Only the anatomical distance ratio (LCCA-IA)/(LSCA-IA) can independently predict recoarctation in the long term.

Conclusion: There is a strong association between the anatomical distance LCCA-IA and recoarctation. This novel parameter is the only anatomical independent predictor of recoarctation.

Background: Medical advances have extended the lives of adults with CHD, but transitioning to adult healthcare remains challenging. Identifying factors related to lapses in care is crucial for adult females due to their unique healthcare needs.

Objective: We examined the relationships among length of lapses in primary care and cardiology services with quality of life and perceived health status for adult females with CHD.

Methods: A convergent mixed-methods design was used to examine thirty adult females with CHD, aged 21-30. Descriptive statistics and correlational analysis were used to examine perceived health status and quality of life based on lapse of care durations. Thematic analysis was used for qualitative data.

Results: A 12-month or greater lapse in care occurred in 46.7% of participants for cardiology and 60% for primary care, and 30.0% lacked an established adult primary care provider. Participants cited healthcare system barriers, provider shortages, and difficult transitions from paediatric providers as contributing factors to lapses in care. Despite lapses, most participants reported similar or better physical health scores than the general population. However, those with either no or prolonged lapse in cardiology care had lower mental health scores. Participants with primary care lapses greater than 6 months reported better quality-of-life scores than those with uninterrupted care.

Conclusion: Significant gaps, which contribute to lapses of care, persist in transition care for females with CHD. Findings highlight the need for structured transition planning, improved primary care access, and an integrated primary-specialty care model to improve transition.