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Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.

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Aim: The objective of this study was to evaluate the healthcare costs and resource utilization of pediatric pulmonary arterial hypertension management at a third-level hospital in Mexico. Methods: A retrospective cohort study was conducted in a pediatric population with pulmonary arterial hypertension. Only direct medical costs, derived from pharmacological treatment, laboratory tests, physician visits and hospitalizations, were considered. From an institutional perspective, all costs were accounted for in 2019 US dollars. Results: A total of 82 patients were included. Of these, 55% were female and the mean age was 6.9 (standard deviation ± 4) years. The mean annual cost was $17,452.14 (standard deviation ± $38,944.10), with a median cost of $8832.75. Conclusion: Pulmonary arterial hypertension is a costly disease, with hospitalization and pharmacological treatment being areas with a higher economic burden. Functional class IV has greater resource utilization and costs.

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We present an institutional guide for a referral to the specialized care center and initial management of pediatric patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with severe manifestations of pediatric inflammatory multisystemic syndrome or symptoms similar to Kawasaki syndrome, and who must have a multidisciplinary approach to ensure adequate treatment and safety for the team of Health.

Presentamos una guía para la referencia al centro de atención especializada y el manejo inicial de pacientes pediátricos infectados por el coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2) con manifestaciones graves del síndrome multisistémico inflamatorio pediátrico o síntomas semejantes al síndrome de Kawasaki y que deben tener un abordaje multidisciplinario para garantizar un adecuado tratamiento y la mayor seguridad para el equipo de salud.

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BACKGROUND: Familial hypercholesterolemia (FH) remains underdiagnosed and undertreated. OBJECTIVE: Report the results of the first years (2017-2019) of the Mexican FH registry. METHODS: There are 60 investigators, representing 28 federal states, participating in the registry. The variables included are in accordance with the European Atherosclerosis Society (EAS) FH recommendations. RESULTS: To date, 709 patients have been registered, only 336 patients with complete data fields are presented. The mean age is 50 (36-62) years and the average time since diagnosis is 4 (IQR: 2-16) years. Genetic testing is recorded in 26.9%. Tendon xanthomas are present in 43.2%. The prevalence of type 2 diabetes is 11.3% and that of premature CAD is 9.8%. Index cases, male gender, hypertension and smoking were associated with premature CAD. The median lipoprotein (a) level is 30.5 (IQR 10.8-80.7) mg/dl. Statins and co-administration with ezetimibe were recorded in 88.1% and 35.7% respectively. A combined treatment target (50% reduction in LDL-C and an LDL-C <100 mg/dl) was achieved by 13.7%. Associated factors were index case (OR 3.6, 95%CI 1.69-8.73, P = .002), combination therapy (OR 2.4, 95%CI 1.23-4.90, P = .011), type 2 diabetes (OR 2.8, 95%CI 1.03-7.59, P = .036) and age (OR 1.023, 95%CI 1.01-1.05, P = .033). CONCLUSION: The results confirm late diagnosis, a lower than expected prevalence and risk of ASCVD, a higher than expected prevalence of type 2 diabetes and undertreatment, with relatively few patients reaching goals. Recommendations include, the use of combination lipid lowering therapy, control of comorbid conditions and more frequent genetic testing in the future.

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Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

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Resumen Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Abstract Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

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Abstract: Objective: REMEHIP is a prospective, multicentre registry on pulmonary hypertension. The main objective will be to identify the clinical profile, medical care, therapeutic trends and outcomes in adult and pediatric Mexican patients with well-characterized pulmonary hypertension. Methods: REMEHIP a multicenter registry began in 2015 with a planned recruitment time of 12 months and a 4-year follow-up. The study population will comprise a longitudinal cohort study, collecting data on patients with prevalent and incident pulmonary hypertension. Will be included patients of age >2 years and diagnosis of pulmonary hypertension by right heart catheterization within Group 1 and Group 4 of the World Health Organization classification. The structure, data collection and data analysis will be based on quality current recommendations for registries. The protocol has been approved by institutional ethics committees in all participant centers. All patients will sign an informed consent form. Currently in Mexico, there is a need of observational registries that include patients with treatment in the everyday clinical practice so the data could be validated and additional information could be obtained versus the one from the clinical trials. In this way, REMEHIP emerges as a link among randomized clinical trials developed by experts and previous Mexican experience.

Resumen: Objetivo: REMEHIP es un registro prospectivo, multicéntrico en hipertensión pulmonar. El objetivo principal será identificar el perfil clínico, atención médica, tendencias terapéuticas y evolución en pacientes mexicanos adultos y pediátricos con hipertensión pulmonar bien caracterizada. Métodos: El REMEHIP comenzó en el año de 2015 y se planea un reclutamiento de 12 meses con un seguimiento de 4 años. La población en estudio será una cohorte longitudinal y se obtendrán datos de pacientes prevalentes e incidentes con hipertensión pulmonar. Se incluirán pacientes con edad > 2 años con diagnóstico de hipertensión pulmonar demostrado por cateterismo cardiaco derecho del Grupo 1 y Grupo 4 de la clasificación de la Organización Mundial de la Salud. La estructura, colección de datos y el análisis se establecerá a través de las recomendaciones actuales de calidad para los registros. El protocolo ha sido aprobado por los comités de ética de todos los centros participantes. Todos los pacientes firmarán un consentimiento informado. Actualmente en México existe una necesidad de registros observacionales que incluyan a pacientes con tratamiento en la práctica clínica cotidiana, de tal forma que los datos obtenidos podrían validarse y el resto de la información podría compararse con la derivada de los estudios clínicos. De esta forma REMEHIP surge como un vínculo entre los estudios clínicos aleatorizados conducidos por expertos y la experiencia mexicana previa.

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