International journal of cardiology. Congenital heart disease最新文献

Compression of left main coronary artery is a severe complication in PAH and the treatment strategy is tricky and should be individualized. We presented a patient with large atrial septal defect (ASD) and non-Eisenmenger pulmonary arterial hypertension (PAH). Right heart catheterization (RHC) showed the defect was non-correctable due to a pulmonary vascular resistance (PVR) of 9.49 Wood Units. EKG suggested myocardial ischemia and coronary angiography and coronary CTA confirmed proximal left main coronary artery was compressed by extremely dilated pulmonary arterial trunk, which is named left main coronary compression syndrome (LMCS). Percutaneous coronary intervention (PCI) using drug-eluting stent was performed and combined PAH-specific medications was prescribed simultaneously. At 6-month following the PCI procedure, PVR had decreased to 3.43 Wood Units, and percutaneous ASD closure procedure was performed. A 6-month follow-up the patient was asymptomatic and had significantly improved pulmonary hemodynamics. LMCS is rare among patients with ASD-PAH. PCI combined with PAH-specific medications allowed the closure of ASD, resulting in clinical improvement.

The increasing population of adult patients with congenital heart disease (ACHD) is at risk of developing coronary artery disease (CAD) and other atherosclerotic cardiovascular diseases due to exposure to cardiovascular risk factors. The impact of this exposure is growing larger as life expectancy of these subjects increases with the progressive improvement in management of congenital heart disease. Studies have shown that ACHD patients have a higher risk for CAD than their non-ACHD matches, highlighting the need for awareness and prevention efforts among congenital heart disease specialists and non-ACHD cardiologists. At the same time, ACHD patients with CAD often present specific characteristics all practicing cardiologists should be aware of. While further research is needed to fully understand the mechanisms underlying the higher CAD risk in this population, this article summarizes key evidence on CAD in ACHD and emphasizes on one hand the importance of early screening and management of known cardiovascular risk factors in ACHD patients, particularly those who are younger, female, or have more complex/severe CHD. On the other hand, it calls for a broader knowledge of ACHD risk for CAD and its peculiarities among all cardiologists.

A case of unilateral vascular hypoplasia is presented. A female patient was born with a complex aortic arch anatomy - a double aortic arch with an interrupted left arch. Surgical correction was performed at the age of 3 months. The patient was also noted to have had an ipsilateral large infantile haemangioma. These findings raised the suspicion of the diagnosis of PHACE syndrome. PHACE syndrome is an acronym for Posterior fossa abnormalities, Haemangioma, Arterial anomalies, Cardiac anomalies and Eye anomalies. Future research is needed to elucidate the underlying pathophysiology in PHACE syndrome.

Congenital heart disease (CHD) often involves the systemic right ventricle (SRV), which is the morphological right ventricle that supports systemic circulation. SRV patients are at a higher risk of sudden cardiac death (SCD) than other adult CHD patients and continues to be a significant cause of death in this aging population. However, the pathophysiology of ventricular arrhythmias in SRV is still not fully understood, and there may be differences between subtypes of CHD. Although these events are rare, predicting them is challenging. This review discusses contemporary strategies for assessing and preventing the risk of ventricular arrhythmias in SRV patients. Several risk factors have been identified to be associated with ventricular arrhythmias in patients with SRV. A recent risk stratification model combines independently associated factors into a risk score, and subpulmonary left ventricle dysfunction is emerging as a critical factor in risk assessment. Cardiac magnetic resonance imaging, biomarkers, and genetic data may refine the ability to predict ventricular arrhythmias in SRV. However, the question of whether implantable cardioverter-defibrillators (ICDs) should be used as a preventive measure in this cohort remains unanswered. Multicenter studies are needed to evaluate risk models and ICD use in this aging population. Given that ICDs have drawbacks, such as a high rate of inappropriate shocks and late lead-related complications, shared clinical decision-making is crucial when considering their use. The review emphasizes the need for further research in this area to improve the identification of patients at risk of clinical ventricular arrhythmias and to develop effective prevention strategies.

Background

In adult patients, after a Fontan procedure, high central venous pressure (CVP) is a hemodynamic risk factor associated with poor prognosis. High liver stiffness (LS) on transient elastography (TE) is associated with high CVP in patients with heart failure without liver disease. Here, we investigated whether LS assessment using TE is a reliable method to noninvasively evaluate CVP in adult patients after a Fontan procedure, who can present varying degrees of liver fibrosis as a complication.

Methods

We measured LS using TE and CVP by cardiac catheterization in 24 adult patients who had undergone a Fontan procedure. The estimated CVP was calculated using the previously reported formula: −5.8 + 6.7 × ln[LS]. We examined the correlation between LS and CVP, and degree of agreement between the estimated and measured CVPs. Patients were divided into two groups, with or without suspected liver cirrhosis, based on abdominal imaging studies.

Results

The median patient age was 35 years (interquartile range 25, 39). Overall, there was a strong correlation between LS and CVP (ρ = 0.83, p < 0.001). The estimated CVP based on LS and the CVP measured using cardiac catheterization were positively correlated; however, the estimated CVP tended to be higher than the measured CVP (mean difference 0.9 mmHg [95% limits of agreement: −2.8 to 4.6 mmHg]). These results were consistent across all groups.

Conclusions

In adult patients after a Fontan procedure, LS measured by TE showed a positive correlation with CVP by cardiac catheterization. TE can be useful as a noninvasive estimation of CVP.

Patients with repaired Tetralogy of Fallot (rTOF) have risks of late life-threatening sequelae, including right ventricular (RV) dilation and failure, arrhythmias, and sudden death. QRS prolongation is a well-known ECG predictor of these outcomes but has poor sensitivity for mortality. Growing evidence demonstrates QRS fragmentation (fQRS) as a better prognostic marker for mortality in adults with rTOF, though the two markers have not been directly compared as correlates for CMR abnormalities. Additionally, fQRS has never been studied in pediatric TOF. This single institution retrospectively reviewed 138 CMRs in rTOF patients (median age 21.7 years) who had a corresponding 12-lead ECG within 1 year. fQRS was defined as ≥3 R-waves/notches in the R/S complex (>2 in right bundle branch block) in ≥2 contiguous leads. QRS prolongation was defined as QRS ≥160 ms. Nearly half (46%) the sample had fQRS (42.1% of pediatric subgroup), and 26% had QRS prolongation. Both markers were significantly associated with reduced RV ejection fraction (EF%) (p < 0.01) and larger RV end-diastolic volumes (p < 0.01). QRS prolongation alone predicted lower LV EF% (p = 0.02). Regression analyses showed both QRS prolongation (p < 0.01) and fQRS (p < 0.01) independently associated with reduced RV EF%; QRS prolongation alone predicted RV dilation (p < 0.01). We concluded that both QRS prolongation and fQRS are equivalent as significant markers of RV dysfunction in rTOF patients. QRS prolongation may be a better surrogate for RV dilation specifically. fQRS was frequently seen in children with rTOF and was significantly associated with similar late structural sequelae.

Developments in medical and surgical techniques have improved survival in women with congenital heart disease (CHD) with most now surviving well into adulthood. Reproductive health amongst these women is underexplored and needs more attention. Women with CHD are known to have more menstrual dysfunction than the general population and have higher maternal and fetal risks when they become pregnant. Adequate and timely preconception counselling, including contraception within a multidisciplinary team (MDT) are essential to optimise pre pregnancy cardiac status and improve pregnancy outcomes. Counselling regarding fertility, sexuality, contraception and menopause is necessary and should start early, around 12–13 years, and as they transition into adult services. Fertility seems to be reduced in women with complex CHD and consideration for assisted reproduction technique (ART) should be assessed by the MDT as risks associated with ART including ovarian hyperstimulation syndrome, bleeding, thrombosis and infection can have profound effects on women with complex cyanotic CHD.

Background

Thyroid dysfunction may have adverse effects on Fontan hemodynamics. Data on thyroid function in pediatric Fontan patients with or without protein-losing enteropathy (PLE) are limited.

Methods

This retrospective multicenter study included 67 Fontan patients (median age 10.9 years; 35.8% female; 28.4% PLE) in whom thyroid function testing was performed.

Results

Subclinical hypothyroidism (SHT) was present in 16 (23.9%) patients. Subjects with SHT had significantly lower systolic blood pressure (p = 0.014) and body weight z-score (p = 0.006), were in a worse New York Heart Association (NYHA) functional class (p = 0.004), were more often pacing dependent (p = 0.007), and were more likely to have PLE (p = 0.033, 8/19 (42.1%) patients). Serum thyroid stimulating hormone (TSH) levels were significantly higher in patients with NYHA class ≥ II (p = 0.005), significant atrioventricular valve regurgitation (p = 0.023), elevated serum natriuretic peptides (p = 0.031), and in those with PLE (p = 0.002). Patients with active PLE had significantly higher TSH levels than those in remission (p = 0.003). A strong inverse relationship was found between lower free triiodothyronine (fT3) levels and natriuretic peptides (r: −0.599, p = 0.040). Using binary logistic regression analysis we found that worse NYHA class was an independent predictor of SHT (OR 4.2; 95% CI 1.1–16.1, p = 0.036).

Conclusions

Subclinical thyroid dysfunction is common in Fontan, particularly in patients with hemodynamic derangements and PLE. Future studies are needed to address the prognostic implications of thyroid dysfunction in the Fontan population.