Long-Term Follow-Up After Ablation of Intra-Atrial Re-Entrant Tachycardia in Patients With Congenital Heart Disease: Types and Predictors of Recurrence.

OBJECTIVES: 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). BACKGROUND: IART increases morbidity and mortality in CHD patients. Radiofrequency catheter ablation has evolved into the first-line treatment of this complication. METHODS: 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). RESULTS: 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). CONCLUSIONS: 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.

Intra-atrial re-entrant tachycardia in patients with congenital heart disease: factors associated with disease severity.

Aim: Intra-atrial re-entrant tachycardia (IART) is a common complication in patients with congenital heart disease (CHD) and is related to increased morbidity and mortality. Few reports have been published about factors associated to IART severity. The aim of this study is to analyse factors associated to severe clinical presentation of IART. Methods and Results: Observational study of all consecutive CHD patients who underwent a first IART ablation from January 2009 to December 2015 (94 patients, 39.4% female, and age: 36.55 ± 14.9 years). Severe clinical presentation was defined as heart failure, syncope, shock, electromechanical dissociation (EMD), or aborted sudden death. The majority of patients had moderately or highly complex cardiac defect (90.4%). Types of IART included cavotricuspid isthmus(CTI) dependent in 51% (48), non-CTI-related in 22.3% (20), and both types in 27.7% (26). In 38 patients (40.4%), a severe event occurred and in 16 (17%), the symptoms included shock, syncope, sudden death, or EMD. In 21 (22.3%), severe symptoms were the first manifestation of IART. In multivariate analysis, transposition of the great arteries (TGA) with right systemic ventricle (OR 5.32, 95% C.I. 1.6-7.02, P = 0.0005) and severe dilation of the venous atrium (VsA) (OR 4.17; 95% CI 1.4-8.12, P = 0.0009) were factors independently associated with severity. Conclusion: In our series of 94 CHD patients with a high proportion of moderately to highly complex cardiac defects, severe consequences of IART were frequent. Transposition of the great arteries with systemic right ventricle and severe dilation of VsA were independently associated to severity. Early invasive procedures should be considered for these high-risk patients.

Valvuloplastia aórtica pediátrica: estudio de variables con influencia en los resultados a largo plazo / Pediatric balloon aortic valvuloplasty. Long-term prognostic factors

Fundamentos y objetivo: En la edad pediátrica, la valvuloplastia aórtica es considerada como una opción para el tratamiento paliativo de la estenosis aórtica congénita. Se revisaron las valvuloplastias aórticas sobre válvula nativa realizadas consecutivamente en nuestro centro desde marzo de 1994 hasta junio de 2013, con el objetivo de identificar variables asociadas a necesidad de reintervención en el seguimiento. Métodos: Análisis de pacientes sometidos a valvuloplastia aórtica, en relación a sus características clínicas, ecocardiográficas y evolutivas, de una serie de 51 individuos, dividida en dos grupos -neonatal y pediátrico- según si la valvuloplastia se hubiere realizado antes o después del mes de vida. El tiempo mediano de seguimiento fue de 5,8 años (0,5-12,3) por paciente. Resultados: De los 51 pacientes, 20 presentaban alguna malformación cardiaca asociada. Tras el procedimiento la media de los gradientes pico-pico descendió de 60 ± 18 a 25 ± 13 mmHg. Evolutivamente, la valvuloplastia inicial fue suficiente en 24 (47%) enfermos, en 12 (23,5%) pacientes se requirió revalvuloplastia por reestenosis a un tiempo mediano de 0,61 años (0,3-3,2), y en 13 (25%) se indicó cirugía a un tiempo medio de 1,62 años (0,4-10,9); en dos casos (4%) se perdió el seguimiento. Las variables: grupo neonatal, patrón diastólico restrictivo, z-score del diámetro telediastólico ventricular izquierdo < -2,5, gradiente hemodinámico pico-pico > 60 mmHg, se asociaron a necesidad de cirugía (p < 0,05) en el seguimiento. No se encontraron factores asociados a revalvuloplastia. Conclusiones:La valvuloplastia aórtica pediátrica es una opción terapéutica paliativa eficaz. En este estudio las variables descritas anteriormente se asociaron a necesidad de cirugía.

Introduction and objective: Balloon aortic valvuloplasty is regarded as a good palliative option for congenital aortic stenosis in children. Our main objective was to review the results of all consecutives balloon aortic valvuloplasties performed in children over a native valve from march 1994 until June 2013, trying to identify those variables associated with need of reintervention during follow-up. Methods: We studied the echocardiographic, clinical and evolutionary features of 51 pediatric patients who underwent a balloon aortic valvuloplasty. Two groups were recognized: neonatal (n = 11) and pediatric (n = 40) patients depending on whether valvuloplasty was performed before or after the first month of life. Median follow-up was 5.8 years (0.5-12,3). Results: There were 37 boys and 14 girls, and 20 patients had some additional cardiac malformation. The average peak to peak hemodynamic gradient fell from 60 ± 18 a 25 ± 13 mmHg. During follow-up, 12 patients needed a second valvuloplasty for reestenosis (23.5%) at 0.61 years (0.3-3.2) and 13 others needed surgery (25%) at 1.62 years (0.4-10.9); 2 cases (4%) had no follow-up. The variables neonatal group, echocardiographic restrictive diastolic pattern, z-score of end-diastolic left ventricle diameter < -2.5, and peak to peak hemodynamic gradient > 60 mmHg were associated with need of surgery during follow-up (p < 0.05). However, we did not find factors associated with repeated valvuloplasty. Conclusions: Balloon aortic valvuloplasty in children is an effective option for palliative treatment of congenital aortic stenosis. In this study the variables previously described were predictors of the need of surgery.

"Pulmonary valve replacement diminishes the presence of restrictive physiology and reduces atrial volumes": a prospective study in Tetralogy of Fallot patients.

Pulmonary valve replacement (PVR) reduces right ventricular (RV) volumes in the setting of long-term pulmonary regurgitation after Tetralogy of Fallot (ToF) repair; however, little is known of its effect on RV diastolic function. Right atrial volumes may reflect the burden of RV diastolic dysfunction. The objective of this paper is to evaluate the clinical, echocardiographic, biochemical and cardiac magnetic resonance (CMR) variables, focusing particularly on right atrial response and right ventricular diastolic function prior to and after elective PVR in adult patients with ToF. This prospective study was conducted from January 2009 to April 2013 in consecutive patients > 18 years of age who had undergone ToF repair in childhood and were accepted for elective PVR. Twenty patients (mean age: 35 years; 70% men) agreed to enter the study. PVR was performed with a bioporcine prosthesis. Concomitant RV reduction was performed in all cases when technically possible. Pulmonary end-diastolic forward flow (EDFF) decreased significantly from 5.4 ml/m(2) to 0.3 ml/m(2) (p < 0.00001), and right atrial four-chamber echocardiographic measurements and volumes by 25% (p = 0.0024): mean indexed diastolic/systolic atrial volumes prior to surgery were 43 ml/m(2) (SD+/-4.6)/63 ml/m(2) (SD+/-5.5), and dropped to 33 ml/m(2) (SD+/-3)/46 ml/m(2) (SD+/-2.55) post-surgery. All patients presented right ventricular diastolic and systolic volume reductions, with a mean volume reduction of 35% (p < 0.00001). Right ventricular diastolic dysfunction was common in a population of severely dilated RV patients long term after ToF repair. Right ventricular diastolic parameters improved as did right atrial volumes in keeping with the known reduction in RV volumes, after PVR.

Cierre percutáneo de comunicaciones interauriculares en pacientes con cirugía de switchauricular para la D-transposición de grandes arterias / Percutaneous closure of baffle leaks in patients with atrial switch operation for d-transposition of the great arteries

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Usefulness of progesterone-only components for contraception in patients with congenital heart disease.

The objective of the study is to report the contraceptive methods used by patients with congenital heart disease (CHD) before referral to a specific preconception clinic and evaluate safety and treatment adherence of the alternative contraception method, progesterone-only component (PC), offered. Contraceptive methods in the CHD population reported included estrogen-progesterone combined contraceptives (EPCC), despite the potential risk for thromboembolism. PC has been suggested as an alternative, but, no information on its use has been reported. Retrospective analysis was performed of all patients (n = 237) referred to the preconception clinic of an adult CHD center. Thirty-three percent of patients had used EPCC in the past; 3.8% had had thromboembolic events during its use. Current contraception consisted of barrier methods in 58% of patients, EPCC in 18%, and PC in 1.3%; 21.7% of patients were not using any contraception. PC was offered as an alternative in 146 patients; 73% of patients agreed to start the treatment. At a median follow-up of 1 year, 73% of patients who started PC maintained the treatment. Gynecologic side effects were reported in 25% of patients, with no cardiovascular effects. In conclusion, a significant proportion of patients with CHD were former users of EPCC, although some had formal contraindications, and the rate of PC use before referral to the preconception clinic was low. After being offered as an alternative treatment, the use of PC in its various forms was extensive, with no thrombogenic side effects and an acceptable rate of gynecologic side effects being reported.

Congenital left ventricular subaortic aneurysm in an asymptomatic child.

A newborn was referred to our department, due to the presence of a cardiac murmur. Echocardiographic study showed a small membranous ventricular septal defect and a yuxta-aortic mass. In the follow-up, patient remained asymptomatic, ventricular septal defect closed, and yuxta-aortic mass increase in size with somatic growth. A transesophageal echocardiography was carried out, showing a fibrotic and dyskinetic left ventricular subaortic aneurysm of 19×18 mm. Given that the patient remained asymptomatic, a conservative approach was adopted. Congenital left ventricular subaortic aneurysm is an infrequent entity, and rupture, endocarditis, coronary compression, thromboembolism, arrhythmias and aortic insufficiency are some of its complications, but, as in our patient, if aneurysm is not associated with complications, patient is asymptomatic, and aneurysm growth is not very important, a conservative approach can be adopted, with a close surveillance.