Myocardial fibrosis resulting from the excessive deposition of collagen fibers through the myocardium is a common histopathologic finding in a wide range of cardiovascular diseases, including ...congenital anomalies. Interstitial fibrosis has been identified as a major cause of myocardial dysfunction since it distorts the normal architecture of the myocardium and impairs the biological function and properties of the interstitium. This review summarizes current knowledge on the mechanisms and detrimental consequences of myocardial fibrosis in heart failure and arrhythmias, discusses the usefulness of available imaging techniques and circulating biomarkers to assess this entity and reviews the current body of evidence regarding myocardial fibrosis in the different subsets of congenital heart diseases with implications in research and treatment.
Right ventricular (RV) resynchronization therapy (CRT) with stimulation electrode implantation in the latest activation area guided by a high‐density electroanatomic activation map was successfully ...performed in a patient with repaired tetralogy of Fallot (rToF), severe pulmonary regurgitation (PR), and severe dysfunction and dilatation of the right ventricle. An improvement in his clinical condition and intraventricular synchrony was achieved. There is a mechanical‐electrical interaction in the right ventricle of patients with rToF; therefore, RV CRT in selected cases may be beneficial. Electroanatomic activation mapping can help achieve maximum clinical benefit by identifying the optimal stimulation site.
AbstractIntroduction and objectivesAlthough pulmonary valve stenosis (PVS) is considered a low risk congenital heart disease, there have been reports of complications and the need for reintervention ...throughout follow-up. The aims of this study were to evaluate the long-term outcome of repaired PVS and to identify predictors of cardiovascular complications and reintervention.MethodsWe studied 158 adult patients with repaired PVS (repair procedures performed from 1957 to 2010) receiving active follow-up in a tertiary referral center.ResultsA total of 95 patients (60%) received surgical treatment, and 63 patients (40%) received percutaneous pulmonary balloon valvuloplasty. At the end of follow-up (27 years, IQR, 20-33 years), most patients (n = 134, 84.8%) were in New York Heart Association functional class I, but 61 patients (38.6%) required a reintervention, mainly pulmonary valve replacement (17.7%, n = 28), and 19 patients (12%) had at least one cardiovascular complication: 13 (8.2%) supraventricular arrhythmias, 6 (3.8%) heart failure, 5 (3.2%) stroke, 1 (0.6%) death, 1 (0.6%) thromboembolism, and 1 (0.6%) ventricular arrhythmia. Multivariate analysis showed that age at PVS repair (HR, 1.08; 95%CI, 1.04-1.12; P < .001) and the presence of cyanosis before PVS repair (HR, 5.23; 95%CI, 1.99-13.78; P= .001) were independent predictors for cardiovascular complications. ConclusionsGood long-term outcome can be expected after PVS repair, but complications and the need for reintervention may appear. Older age and the presence of cyanosis at PVS repair emerged as predictors of cardiovascular complications and identified a population that may merit stricter control.
Echocardiography (Echo) has been used to define restrictive physiology as any presence of end-diastolic forward flow (EDFF) on the pulmonary artery, despite constant small amount of EDFF is present ...in healthy children, being values lesser than in children with repaired right ventricular outflow tract obstructive lesions (RVOT). No information regarding EDFF quantitative assessment has been made in adult healthy controls (HC).
81 consecutive patients (mean age: 37 years; 53% men) with RVOT lesions were studied. Forty-three age-and-sex matched HC were evaluated. Echo parameters were: EDFF peak velocity in inspiration (EDFF_i), in expiration (EDFF_e), EDFF_i/EDFF_e ratio, maximum velocity-time integral EDFF (VTI_EDFF).
A small constant EDFF was present in HC. Mean values of EDFF_i, EDFF_e, VTI-EDFF were significantly lower in HC compared to patients: 26 cm/s (SD 6) vs 49.6 cm/s (SD ±22), 25 cm/s (SD ±6) vs 40 cm/s (SD ±19), 2.3 cm ( ±0.6) vs 6.2 cm ( ±3.5), respectively, p < 0.001. The EDFF_i/EDFF_e ratio was 1 ( ±0.13) in HC vs 1.25 ( ±0.25) in patients p < 0.001. Based on their normal distribution in HC, we propose cut-off values > 2SD for the different parameters. VTI-EDFF could easily be used as a non-invasive parameter to distinguish patients from HC and provide a new definition of RP; the presence of respiratory variation should be considered a key factor to differentiate HC from patients. In conclusion, any amount of EDFF throughout the respiratory cycle is not useful for identifying abnormal EDFF. A new approach based on cut-off values obtained from HC and quantitative EDFF assessment is proposed to provide a new definition of abnormal EDFF.
The aim of this study was to analyze the long-term outcomes after intra-atrial re-entrant tachycardia (IART) ablation in congenital heart disease (CHD).
IART increases morbidity and mortality in CHD ...patients. Radiofrequency catheter ablation has evolved into the first-line treatment of this complication.
This was a prospective, single-center study of all consecutive CHD patients who underwent first ablation for IART from January 2009 to December 2015 (n = 94, 39.4% female, age 36.55 ± 14.9 years, follow-up 44.45 ± 22.7 months).
During the study period, 130 procedures were performed (n = 94, 1.21 ± 0.41 IART/patient). In the first procedure, 114 IART were ablated (short-term success 74.66%). Forty-nine percent of the patients whose IART was ablated had non-cavotricuspid isthmus (CTI)-related IART (alone or with concomitant CTI IART). After the first ablation, 54.3% maintained sinus rhythm (SR), 23.9% presented with recurrence of the ablated IART, 14.2% developed new IART, and 7.6% presented with atrial fibrillation (AF). After the second radiofrequency catheter ablation, 78.3% were in SR, 8.7% presented with AF, and 23.0% presented with IART (50% new IART). Multivariate predictors of recurrences were non-CTI IART (hazard ratio HR: 5.06; 95% confidence interval CI: 1.6 to 15.9; p = 0.006), PR interval >200 ms (HR: 4.02; 95% CI: 1.9 to 11.3; p = 0.009), AF induction (HR: 3.11; 95% CI: 1.1 to 9.1; p = 0.04). and previous AF (HR: 3.08; 95% CI: 1.1 to 9.3; p = 0.04). A risk score according multivariate model identified 3 levels of recurrence risk: 5.8%, 20%, and 58.5% (area under the receiver-operating characteristic curve 0.8 ± 0.03; p < 0.0001).
Ablation of IART in CHD is a challenging procedure, but after ablation in experienced centers, SR can be maintained in 78.3%. Predictors of recurrences are non-CTI-related IART, long PR interval, and previous or induced AF. A risk score based on these factors can be useful for recurrence prediction.
There is little information of progestogen-only contraceptives in patients with congenital heart disease (CHD) on the long-term.
To evaluate the use of contraception in patients with CHD. We studied ...both short-acting reversible contraceptives (SARCs), oral progestin-only pills (POPs) and long-acting reversible contraceptives (LARCs): intrauterine devices (IUD-IPs) and subdermal implants both impregnated with progestogens (SI-IPs).
Retrospective study of all women attending the preconception clinic. Contraceptive methods were classified in three TIERs of effectiveness before and after consultation. ESC classification regarding pregnancy risk, WHOMEC classification for combined oral contraceptive safety was collected.
Six hundred and fifty-three patients. A significant proportion of them switched from TIER 3 to TIER 2 or 1 (
< .001) after consultation. One hundred and ninety-nine patients used POPs, 53 underwent IUD-IPs implantation and 36 SI-IPs, mean duration was 58 ± 8, 59 ± 8 and 53 ± 38 months, respectively.
Because of their safety and efficacy, IUD-IPs and SI-IPs should be considered as first-line contraception in patients with CHD.
Background
Intra‐atrial re‐entrant tachycardia (IART) in patients with congenital heart disease (CHD) increases morbidity and mortality. Radiofrequency catheter ablation has evolved as the first‐line ...treatment. The aim of this study was to analyze the acute success and to identify predictors of failed IART radiofrequency catheter ablation in CHD.
Methods and Results
The observational study included all consecutive patients with CHD who underwent a first ablation procedure for IART at a single center from January 2009 to December 2015 (94 patients, 39.4% female, age: 36.55±14.9 years). In the first procedure, 114 IART were ablated (acute success: 74.6%; 1.21±0.41 IART per patient) with an acute success of 74.5%. Cavotricuspid isthmus–related IART was the only arrhythmia in 51%; non–cavotricuspid isthmus–related IART was the only mechanism in 27.7% and 21.3% of the patients had both types of IART. Predictors of acute radiofrequency catheter ablation failure were as follows: nonrelated cavotricuspid isthmus IART (odds ratio 7.3; confidence interval CI, 1.9–17.9; P=0.04), previous atrial fibrillation (odds ratio 6.1; CI, 1.3–18.4; P=0.02), transposition of great arteries (odds ratio, 4.9; CI, 1.4–17.2; P=0.01) and systemic ventricle dilation (odds ratio 4.8; CI, 1.1–21.7; P=0.04) with an area under the receiver operating characteristic curve of 0.83±0.056 (CI, 0.74–0.93, P=0.001). After a mean follow‐up longer than 3.5 years, 78.3% of the patients were in sinus rhythm (33.1% of the patients required more than 1 radiofrequency catheter ablation procedure).
Conclusions
Although ablation in CHD is a challenging procedure, acute success of 75% can be achieved in moderate–highly complex CHD patients in a referral center. Predictors of failed ablation are IART different from cavotricuspid isthmus, previous atrial fibrillation, and markers of complex CHD (transposition of great arteries, systemic ventricle dilation).
To describe the aortic-related risks associated with pregnancy in women with bicuspid aortic valve (BAV) and to evaluate changes in aortic diameter in pregnancy.
Prospective observational study of ...patients with BAV from a single-site registry of pregnant women with structural heart disease between 2013 and 2020. Cardiac, obstetric and neonatal outcomes were studied. An assessment of aortic dimensions was performed during pregnancy by two-dimensional echocardiography. Aortic diameters were measured at the annulus, root, sinotubular junction and maximum ascending aorta diameter, and the largest diameter was used. Measurements of the aorta were made using the end-diastolic leading edge-to-leading edge convention.
Forty-three women (32.9 years, IQR 29.6-35.3) with BAV were included: 9 (20.9%) had repaired aortic coarctation; 23 (53.5%) had moderate or severe aortic valve disease; 5 (11.6%) had a bioprosthetic aortic valve; and 2 (4.7%) had a mechanical prosthetic aortic valve. Twenty (47.0%) were nulliparous. The mean aortic diameter in the first trimester was 38.5 (SD 4.9) mm, and that in the third trimester was 38.4 (SD 4.8) mm. Forty (93.0%) women had an aortic diameter of <45 mm; 3 (7.0%) had 45-50 mm; and none had >50 mm. Three women (6.9%) with BAV presented cardiovascular complications during pregnancy or the postpartum period (two prosthetic thrombosis and one heart failure). No aortic complications were reported. There was a small but significant increase in aortic diameter during pregnancy (third trimester vs first trimester, 0.52 (SD 1.08) mm; p=0.03). Obstetric complications appeared in seven (16.3%) of pregnancies, and there were no maternal deaths. Vaginal non-instrumental delivery was performed in 21 (51.2%) out of 41 cases. There were no neonatal deaths, and the mean newborn weight was 3130 g (95% CI 2652 to 3380).
Pregnancy in BAV women had a low rate of cardiac complications with no aortic complications observed in a small study group. Neither aortic dissection nor need for aortic surgery was reported. A low but significant aortic growth was observed during pregnancy. Although requiring follow-up, the risk of aortic complications in pregnant women with BAV and aortic diameters of <45 mm at baseline is low.
Intra-atrial re-entrant tachycardia (IART) is a severe complication in patients with congenital heart disease (CHD). Cavotricuspid isthmus (CTI)-related IART is the most frequent mechanism. However, ...due to fibrosis and surgical scars, non-CTI-related IART is frequent. The main objective of this study was to describe the types of IART, circuit locations, and to analyze predictors of CTI versus non-CTI-related IART. This is an observational study that includes all consecutive patients with CHD who underwent a first IART ablation in a single referral tertiary hospital from January 2009 to December 2015 (94 patients; 39.4% women; age: 36.55 ± 14.9 years, 40.4% with highly complex cardiac disease). During the study, 114 IARTs were ablated (1.21 ± 0.41 IARTs per patient). CTI-related IART was the only arrhythmia in 51% (n = 48) of patients; non-CTI-related IART was the only mechanism in 27.7% (n = 26), and 21.3% of patients (n = 20) presented the two types of IART. Severe dilation of the systemic ventricle, absence of severe dilation of the venous atrium, highly complex cardiac defects, and nontypical electrocardiography (ECG) were related to non-CTI-related IART in univariate analysis. In multivariate analysis, nontypical ECG (odds ratio 3.64; 1.01 to 4.9; p = 0.049) and grade III CHD complexity (odds ratio 9.43; 1.44 to 11.7; p = 0.001) were predictors of non-CTI-related IART. In conclusion, in our population with a high proportion of complex CHD, CTI-related IART was the most frequent mechanism, although non-CTI-related IART was present in 49% (alone or with concomitant CTI-related IART). High-grade CHD complexity and nontypical ECG were strongly related to non-CTI IART.
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