Cardiometabolic Risk Factors in Mexican Adults With Congenital Heart Disease.

Background: In recent decades, adults living with congenital heart disease (ACHD) have improved their survival, thus increasing their predisposition to the onset of cardiometabolic risk factors and chronic health conditions. Objectives: The purpose of this study was to describe cardiometabolic risk profiles in the ACHD population and their relationship to congenital heart disease (CHD) lesion complexity. Methods: We performed a cross-sectional study from ACHD in a third-tier referral center in Mexico City. The association between cardiometabolic risk factors and CHD complexity was estimated using logistic regression models. Results: Our study cohort included 1,171 ACHD patients (median age: 31 [IQR: 23.2-42.7] years, male 63.6%). Cardiac diagnosis was classified as mild (44.9%), moderate (37.8%), and severe (17.2%) CHD complexity. Low high-density lipoprotein cholesterol (55%) was the most common cardiometabolic risk factor; followed by insulin resistance (54.5%) and prediabetes (52.4%). Patients with mild and moderate CHD had a higher prevalence of obesity and metabolic syndrome, while patients with severe CHD had a higher prevalence of hyperuricemia and subclinical hypothyroidism. In the logistic regression analysis, the severity of CHD was associated with higher odds of hyperuricemia (moderate CHD, OR: 1.87; 95% CI: 1.20-2.93; P = 0.010; severe CHD, OR: 2.75; 95% CI: 1.64-4.62; P < 0.001) and lower risks of metabolic syndrome (OR: 0.61; 95% CI: 0.41-0.91; P = 0.010), prediabetes (OR: 0.58; 95% CI: 0.42-0.81; P < 0.001), and arterial hypertension (OR: 0.49; 95% CI: 0.33-0.74; P < 0.001) compared with mild CHD complexity. Conclusions: We observed high rates of cardiometabolic risk factors in Mexican ACHD patients and these risk profiles varied by CHD lesion complexity. These results highlight the need for ongoing metabolic health surveillance in the ACHD population.

Transition Readiness in Teens and Young Adults with Congenital Heart Disease: Can We Make a Difference?

OBJECTIVES: To examine changes in transition readiness (knowledge, self-efficacy, self-management) over time and explore factors associated with transition readiness, including psychosocial quality of life (QOL) and health service utilization in teens/young adults with congenital heart disease. STUDY DESIGN: In a multicenter prospective cohort study, 356 patients, age 14-27 years, completed transition readiness and QOL assessments at routine cardiology visits at baseline and 1-year follow-up. RESULTS: Median patient age was 19.8 years at 1.03 years (IQR 0.98-1.24) following baseline transition readiness assessment. Average knowledge deficit scores decreased at follow-up (P < .0001) and self-efficacy scores increased (P < .0001). Self-management scores increased (P < .0001), but remained low (mean 57.7, 100-point scale). Information was requested by 73% of patients at baseline and was associated with greater increase in knowledge at follow-up (P = .005). Increased knowledge (P = .003) and perceived self-efficacy (P = .01) were associated with improved psychosocial QOL, but not health service utilization at follow-up. Patients who preferred face-to-face information from healthcare providers (47%) vs other information sources were more likely to request information (P < .0001). In patients <18 years old, greater agreement between teen and parental perception of teen's knowledge was associated with greater increase in patient knowledge (P = .02) and self-efficacy (P = .003). CONCLUSION: Transition readiness assessment demonstrated improved knowledge, self-efficacy, and self-management at 1-year follow-up in teens/young adults with congenital heart disease. Improved knowledge and self-efficacy were associated with improved psychosocial QOL. Self-management remained low. Supplemental media for conveying information and greater involvement of parents may be needed to optimize transition readiness.

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

To investigate the role of right ventricular free wall strain (RVFWSL) to predict low functional capacity in repaired tetralogy of Fallot (rTOF). We prospectively enrolled 33 patients with rTOF with moderate to severe PR who underwent rest and peak exercise echocardiography on a semisupine cycloergometer. Conventional function and strain imaging parameters of both ventricles were measured. Patients performing < 7 METS were defined to have low functional capacity. Logistic regression was used to identify parameters associated with low functional capacity. Eleven patients (33.3%) had low functional capacity. These patients were shorter (height 155 ± 7 vs 163 ± 9 cm, p = 0.023), more frequently female (27.3 vs 72.7%, p = 0.024) and had history of Blalock-Taussig shunt (45.5 vs 9.1%, p = 0.027). On multivariate analysis RVFWSL was the only predictor of low functional capacity OR 1.39 (CI 95%, 1.06-1.83., p = 0.018) per % change. A RVFWSL < 17% (absolute value) had an AUC of 0.785, sensitivity of 81.8% and specificity of 77.3% to predict low functional capacity. Right ventricular free wall strain is an independent predictor of low functional capacity in repaired tetralogy of Fallot with moderate to severe PR. A value < 17% might be useful in deciding when to perform pulmonary valve replacement, when functional capacity cannot be objectively measured.

Trasplante cardíaco en adultos con cardiopatías congénitas / Heart Transplantation in Adult Patients with Congenital Heart Disease

RESUMEN Introducción: En las últimas décadas los pacientes con cardiopatías congénitas (CC) han presentando nuevos desafíos en el manejo de las complicaciones, tanto de la patología originaria como de las cirugías correctoras que les han permitido llegar a la adultez. Objetivo: Comunicar los resultados y la evolución de los pacientes con CC que hayan sido evaluados para trasplante en un centro de alta complejidad. Se utilizó la base de datos institucional, y se analizaron los datos de 11 pacientes evaluados para trasplante con diversas patologías congénitas. Accedieron al trasplante 5 de ellos con una sobrevida a 1,6 años del 80%. Los pacientes que se hallaban en lista y no se trasplantaron tuvieron una mortalidad del 66%, y los descartados por comorbilidades presentaron una mortalidad del 35%. Conclusión: El trasplante cardíaco en adultos con CC tiene un riesgo periprocedimiento más elevado que para las cardiopatías adquiridas (CA). No obstante, los que sobreviven el primer año postrasplante tienen una excelente evolución a largo plazo.

ABSTRACT Background: Over recent decades, congenital heart disease (CHD) patients have posed new challenges in the management of complications, both of the original condition as of the corrective surgeries that have allowed them to reach adulthood. Objective: The aim of this study was to report the outcomes and evolution of CHD patients who had been evaluated for transplantation in a tertiary care center. Using the institutional database, data from 11 patients with different congenital diseases were evaluated for transplantation. A total of 5 patients underwent transplantation, with a 1.6-year survival rate of 80%. Mortality rate was 66% for patients who were on the waiting list but were not transplanted, and 35% for those who were ruled out due to comorbidities. Conclusion: Heart transplantation in CHD adult patients present a higher periprocedural risk than in patients with acquired heart diseases. However, those who survive the first post-transplant year have an excellent long-term outcome.

Transition Readiness in Adolescents and Young Adults with Heart Disease: Can We Improve Quality of Life?

OBJECTIVES: We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL). STUDY DESIGN: In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated. RESULTS: Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P < .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02). CONCLUSIONS: Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL.

Lessons Learned From a Critical Analysis of the Fontan Operation Over Three Decades in a Single Institution.

BACKGROUND: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. METHODS: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown. RESULTS: A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery ( P < .001) and pulmonary artery reconstruction ( P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown). CONCLUSION: Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.

Hipertensión arterial pulmonar en cardiopatías congénitas del adulto: Impacto y resultados Datos del Registro GUTI-GUCH / Pulmonary arterial hypertension in adult congenital heart disease: Impact and outcome Data from the GUTI-GUCH Registry / Hipertensão arterial pulmonar em cardiopatias congênitas no adulto: Impacto e resultados Dados do Registro GUTI-GUCH

Introducción. La hipertensión pulmonar (HP) es una complicación grave de las cardiopatías congénitas del adulto (CCA), su aparición se relaciona con un marcado aumento de la morbimortalidad de estos pacientes. Objetivo. Analizar las características epidemiológicas clínicas y de tratamiento farmacológico en pacientes con hipertensión arterial pulmonar (HAP) y su impacto en la sobrevida. Materiales y métodos. Estudio observacional retrospectivo. Utilizando el Registro GUTI-GUCH, se analizaron datos globales. Se seleccionaron pacientes con diagnóstico de HAP secundaria a cardiopatía congénita (HAPG1) y sus subgrupos. SG1: síndrome de Eisenmenger, SG2: cortocircuitos de izquierda a derecha, SG3: HAP coincidente con cardiopatía congénita, SG4: HAP en posoperatorio. Análisis estadístico: SPSS 20, test del Chi cuadrado, curva de Kaplan Meier contrastada por Log Rank. Resultados. Incorporados 1604 pacientes con edad media de 32,5 años, el 55,4% (889) eran mujeres. Pacientes con cardiopatía de riesgo para HAP (CRHAP): 70,5% (1131); con HP: 4,2% (67) del global y el 5,8% (67) de CRHAP. El 71,6% (48) de los pacientes con HP fueron mujeres, con edad de 41,7 ± 14,4 años y seguimiento de 33± 30,9 meses. Pertenecían al HAPG1 el 91% (61) representando el 3,8% del registro. Los pacientes con HAPG1 se subdividían en: SG1: 47,5% (29); SG2: 26,2% (16); SG3: 5% (3) y SG4: 21,3% (13). El Grupo HAPG1 recibía tratamiento farmacológico, especifico: 78,7% (48), monoterapia: 57,4% (35) y terapia combinada: 21,3% (13). La mortalidad en el seguimiento para los pacientes con HP fue del 17,9% (12) y del 16,4% (10) para HAPG1. Se observaron diferencias estadísticamente significativas para HP: p=0,000; HAPG1: p=0,000; subgrupos de HAPG1: p=0,000. No se encontraron diferencias con el SG1: p=0,184. Conclusiones. La HP y la HAPG1 afectan negativamente la sobrevida de los pacientes con CCA. La mitad de ellos es portador de síndrome de Eisenmenger que junto al SG3 son los de mejor evolución. Se observó una elevada prevalencia de mujeres.

Introduction. Pulmonary hypertension (PH) is a serious complication of adult congenital heart disease (ACHD), its incidence is associated with a marked increase in morbidity and mortality of these patients. Objective. We analyze the epidemiological characteristics clinical and pharmacological treatment in patients with pulmonary arterial hypertension (PAH) and their impact on survival. Materials and methods. Retrospective observational study, global data were analyzed using the GUTI-GUCH Registry. We selected patients with diagnosis of PAH related to congenital heart disease (PAH-CHD) and its subgroups. SG1: Eisenmenger syndrome, SG2: shunts left to right, SG3: PAH-CHD, SG4: postoperative PAH. Statistical analysis: SPSS 20, Chi square test, Kaplan Meier curve contrasted by Log Rank. Results. We included 1604 patients with a mean age of 32.5 years, 55.4% (889) were women. Patients with heart disease risk for PAH (HDRPAH): 70.5% (1131); PH: 4.2% (67) overall and 5.8% (67) of HDRPAH. And 71.6% (48) patients with PH were women, with age 41.7 ± 14.4 years and follow up of 33 ± 30.9 months. The 91% (61) belonged to PAH-CHD group representing 3.8% of registry. PAH-CHD patients were subdivided into: SG1: 47.5% (29); SG2: 26.2% (16); SG3: 5% (3) and SG4: 21.3% (13). The PAH-CHD group received pharmacological treatment, specific: 78.7% (48), monotherapy: 57.4% (35) and combination therapy: 21.3% (13). The follow-up mortality for patients with PH was 17.9% (12) and 16.4% (10) for PAH-CHD. Statistically significant differences were observed for PH: p=0.000; PAH-CHD: p=0.000; PAH-CHD subgroups: p=0.000. No differences were found with SG1: p=0.184. Conclusions. The HP and PAH-CHD negatively affect the survival of patients with ACHD. Half of them are carriers of Eisenmenger syndrome by the SG3 are better evolution. A high prevalence of women was observed.

Introdução. A hipertensão pulmonar (HP) é uma complicação grave das cardiopatias congênitas no adulto (CCA), sua incidência está associada com um aumento significativo na morbidade e mortalidade desses pacientes. Objetivo. Foram analisadas as características epidemiológicas clínicas e de tratamento medicamentoso em pacientes com hipertensão arterial pulmonar (HAP) e seu impacto na sobrevivência. Materiais e métodos. Estudo retrospectivo observacional. Foram analisados dados globais utilizando o Registry GUTI-Guch. Foram selecionados pacientes com diagnóstico de HAP secundária a cardiopatia congênita (HAPG1) e seus subgrupos. SG1: síndrome de Eisenmenger, SG2: desvio de sangue da esquerda à direita, SG3: HAP coincidente com cardiopatia congênita, SG4: HAP pós-operatória. Análise estatística: SPSS 20, teste do qui-quadrado, curva de Kaplan Meier contrastada pela Log Rank. Resultados. Foram incluídos 1604 pacientes com uma idade média de 32,5 anos, o 55,4% (889) eram mulheres. Pacientes com cardiopatia de risco para a HAP (CRHAP): 70,5% (1131); HP: 4,2% (67) do global e 5,8% (67) de CRHAP. O 71,6% (48) dos pacientes com HP eram mulheres, com idade de 41,7 ± 14,4 anos e seguimento de 33 ± 30,9 meses. O 91% (61) pertenciam ao grupo HAPG1, representando o 3,8% do registro. Os pacientes com HAPG1 foram subdivididos em: SG1: 47,5% (29); SG2: 26,2% (16); SG3: 5% (3) e SG4: 21,3% (13). O grupo HAPG1 recebeu tratamento farmacológico, específico: 78,7% (48), monoterapia: 57,4% (35) e uma terapia combinada: 21,3% (13). A mortalidade no seguimento dos pacientes com HP foi de 17,9% (12) e do 16,4% (10) para HAPG1. Foram observadas diferenças estatisticamente significativas para a HP: p=0,000; HAPG1: p=0,000; subgrupos HAPG1: p=0,000. Não foram encontradas diferenças com SG1: p=0,184. Conclusões. A HP e a HAPG1 afetar negativamente a sobrevivência de pacientes com CCA. A metade deles é portadora da síndrome de Eisenmenger e com o SG3 são os de melhor evolução. Observou-se uma alta prevalência de mulheres.

Double-orifice mitral valve.

We report the case of a rare congenital anomaly, a double-orifice mitral valve, in a 21-year-old woman who was asymptomatic and had no history of heart disease. Transthoracic echocardiography revealed two functionally normal orifices mitral valve of equal size. As the presentation in adulthood is rare, echocardiographers should be trained to make the appropriate diagnosis.

Una causa poco frecuente de hipertrabeculación exclusiva del ventrículo derecho: ventrículo derecho no compacto / An unusual cause of right ventricular hypertrabeculation exclusive of the right ventricle: non compaction of right ventricle

La cardiopatía por falta de compactación ventricular es una entidad infrecuente, que generalmente compromete el ventrículo izquierdo ocasionando falla cardíaca, arritmias ventriculares, embolismo periférico y muerte súbita. Hasta el momento, se han descrito muy pocos casos de ventrículo derecho no compacto, por lo cual no hay criterios diagnósticos establecidos y se desconoce como es la evolución clínica y la respuesta al tratamiento de este grupo de pacientes. Se describe el caso de un paciente con hipertrabeculación marcada del ventrículo derecho en quien se descartó un origen estructural de la misma, haciéndose diagnóstico de ventrículo derecho no compacto.

Non compaction cardiomyopathy is a rare entity which usually involves the left ventricle causing heart failure, ventricular arrhythmias, peripheral embolism and sudden death. So far, very few cases of right ventricular non compaction have been described, for which no established diagnostic criteria exist and the clinical course as well as the response to treatment of these patients remain unknown. We describe the case of a patient with marked right ventricular hypertrabeculation in whom a structural origin of it was discarded, whereby the diagnosis of right ventricular non-compactation was made.

Resolución exitosa de la estenosis congénita de las dos ramas de la arteria pulmonar en un adulto mediante el uso de stents / Successful resolution of congenital stenosis of the two branches of the pulmonary artery in an adult using stents

La estenosis de las arterias pulmonares son rara vez diagnosticadas en edad adulta, convirtiéndose su tratamiento en una compleja situación con diferentes posibilidades terapéuticas. Las causas mas frecuentes corresponden a las adquiridas, siendo las mas común la hipertensión pulmonar trombo embólica crónica, quedando reducido numero de casos congénitos asociado principalmente a síndrome genéticos y malformaciones cardiacas asociadas. El tratamiento quirúrgico convencional puede convertirse en un desafío dadas las condiciones anatómicas y funcionales encontradas en estadios avanzados propias del diagnostico tardío y en especial en el caso presentado el cual esta iniciando la sétima década de la vida. La posibilidad de resolución percutánea de estas lesiones cobra importancia a la luz de la amplia utilización de esta en pacientes pediátricos, implementando el uso cada vez mayor de balones con dilatación percutánea y la utilización de stents cada vez más sostificados con buenos resultados. Se presenta nuestro primer caso de resolución percutánea de lesiones coronarias y de arteria pulmonar bilateral en un paciente previamente programado para cirugía de reparación con parche y by pass coronario, el cual evolucionó en formas satisfactoria recobrando su actividad normal a las pocas horas del procedimiento.

Stenosis of the pulmonary arteries is rarely diagnosed in adulthood, turning their treatment in a complex situationwith different therapeutic possibilities. The most frequent causes are acquired, being the most common chronicthromboembolic pulmonary hypertension, remaining a small number of congenital cases mainly associated to geneticsyndromes or complex cardiac malformations. The standard surgical treatment may be a challenge given the anatomicaland functional conditions found themselves in advanced stages of late diagnosis and especially in the case presented,who is beginning the seventh decade of his life. The possibility of percutaneous resolution of these lesions becomesimportant in light of the widespread use of this option in pediatric patients, implementing and increasing its use withpercutaneous dilatation balloons and stents with increasing sophistication and showing good results. We present ourfirst case of percutaneous resolution of coronary lesions and bilateral pulmonary artery stenosis in a patient previouslyscheduled for patch repair surgery and coronary bypass, which evolved in successfully, gaining normal activity within fewhours of the procedure.

Persistent institutional difficulties in surgery for transposition of the great arteries in guatemala: analysis with the aristotle basic and comprehensive scores.

Background. Neonates with complex congenital cardiac lesions are largely inadequately managed in Guatemala. Methods. Between 1997 and 2009, 79 patients who underwent operations for transposition of the great arteries were identified; 51 (63.3%) had an arterial switch operation (ASO) and 28 (36%) an atrial switch operation (ATSO). The Aristotle Basic Complexity score (ABC score) and the Aristotle Comprehensive Complexity score (ACC score) have been used to aid in the evaluation of quality of care associated with pediatric cardiac surgery by adjusting for operative complexity. Results. In-hospital mortality was 47% for the ASO and 25% for the ATSO group; 36.7% were beyond 1 month of age and many exhibited increased preoperative risk factors. The mean ABC score was 9.75 ± 0.89 and the ACC score was 12.12 ± 2.7, with a mean 2.36-point increase (P < .05). Comparing survivors and nonsurvivors with both scores, significant differences were identified (ABC: P < .04 and ACC: P < .02). Conclusion. During this 13-year period, a low volume of surgery for transposition of the great arteries (TGA) was performed at our institution with a relatively high surgical mortality. Many patients with TGA in Guatemala are either never referred for surgery or referred late. Strategies to improve outcomes for neonates with TGA in Guatemala must include increases in early diagnosis countrywide and prompt referral to our unit. Based on the larger number of neonates with TGA that would be referred to our center, we anticipate that this strategy should substantially improve surgical outcomes and favor overall team-related skills.