Hypertrophic cardiomyopathy. Proposal for a new classification / Miocardiopatía hipertrófica. Propuesta de una nueva clasificación

Abstract Hypertrophic cardiomyopathy (HCM) is a clinical condition, but its name has been subjected to frequent changes over the years, largely because of its morphological and functional heterogeneity, which leads the clinician who is focused on its study to have difficulty in understanding how to diagnose it and when and how to treat it. Regarding its name, it has been called in more than 75 different ways, and it has being classified with difficulty through echocardiography for more than 40 years. Today, it is necessary to understand that the diverse phenotypic behavior, as well as the evolutionary stages of the disease, must be approached in a practical and effective way, so that it easier to understand its clinical behavior and prognosis, as well as the therapeutic needs in each particular case. We review the aspects related to the name of the condition and propose a new classification that could provide the clinical and surgical cardiologist a better understanding of HCM in its various morphological and functional aspects.

Resumen La Miocardiopatía Hipertrófica es una entidad clínica que ha sido sometida durante años a cambios frecuentes en su denominación, en gran parte consecuencia de su heterogeneidad morfológica y funcional, lo que hace que el clínico enfocado a su estudio, tenga dificultad en el entendimiento de cómo hacer el diagnóstico y cuándo y cómo tratarle. Nominativamente ha sido llamada de más de 75 formas diferentes y clasificada con dificultad mediante ecocardiografía hace ya más de 40 años. Hoy en día es necesario entender que su comportamiento fenotípico diverso así como las etapas evolutivas de la enfermedad, deben ser abordadas de una forma práctica y eficaz, de tal forma que ello facilite el entendimiento de su comportamiento clínico y su pronóstico, así como de las necesidades terapéuticas en cada caso en particular. Se hace una revisión de los aspectos nominativos de la entidad y proponemos una nueva clasificación que podría facilitar al cardiólogo clínico y quirúrgico un mejor entendimiento de la Miocardiopatía Hipertrófica en sus diversas formas morfológicas y funcionales.

Hypertrophic cardiomyopathy. Proposal for a new classification.

Hypertrophic cardiomyopathy (HCM) is a clinical condition, but its name has been subjected to frequent changes over the years, largely because of its morphological and functional heterogeneity, which leads the clinician who is focused on its study to have difficulty in understanding how to diagnose it and when and how to treat it. Regarding its name, it has been called in more than 75 different ways, and it has being classified with difficulty through echocardiography for more than 40 years. Today, it is necessary to understand that the diverse phenotypic behavior, as well as the evolutionary stages of the disease, must be approached in a practical and effective way, so that it easier to understand its clinical behavior and prognosis, as well as the therapeutic needs in each particular case. We review the aspects related to the name of the condition and propose a new classification that could provide the clinical and surgical cardiologist a better understanding of HCM in its various morphological and functional aspects.

La Miocardiopatía Hipertrófica es una entidad clínica que ha sido sometida durante años a cambios frecuentes en su denominación, en gran parte consecuencia de su heterogeneidad morfológica y funcional, lo que hace que el clínico enfocado a su estudio, tenga dificultad en el entendimiento de cómo hacer el diagnóstico y cuándo y cómo tratarle. Nominativamente ha sido llamada de más de 75 formas diferentes y clasificada con dificultad mediante ecocardiografía hace ya más de 40 años. Hoy en día es necesario entender que su comportamiento fenotípico diverso así como las etapas evolutivas de la enfermedad, deben ser abordadas de una forma práctica y eficaz, de tal forma que ello facilite el entendimiento de su comportamiento clínico y su pronóstico, así como de las necesidades terapéuticas en cada caso en particular. Se hace una revisión de los aspectos nominativos de la entidad y proponemos una nueva clasificación que podría facilitar al cardiólogo clínico y quirúrgico un mejor entendimiento de la Miocardiopatía Hipertrófica en sus diversas formas morfológicas y funcionales.

Nanostructured Non-Newtonian Drug Delivery Barrier Prevents Postoperative Intrapericardial Adhesions.

With the increasing volume of cardiovascular surgeries and the rising adoption rate of new methodologies that serve as a bridge to cardiac transplantation and that require multiple surgical interventions, the formation of postoperative intrapericardial adhesions has become a challenging problem that limits future surgical procedures, causes serious complications, and increases medical costs. To prevent this pathology, we developed a nanotechnology-based self-healing drug delivery hydrogel barrier composed of silicate nanodisks and polyethylene glycol with the ability to coat the epicardial surface of the heart without friction and locally deliver dexamethasone, an anti-inflammatory drug. After the fabrication of the hydrogel, mechanical characterization and responses to shear, strain, and recovery were analyzed, confirming its shear-thinning and self-healing properties. This behavior allowed its facile injection (5.75 ± 0.15 to 22.01 ± 0.95 N) and subsequent mechanical recovery. The encapsulation of dexamethasone within the hydrogel system was confirmed by 1H NMR, and controlled release for 5 days was observed. In vitro, limited cellular adhesion to the hydrogel surface was achieved, and its anti-inflammatory properties were confirmed, as downregulation of ICAM-1 and VCAM-1 was observed in TNF-α activated endothelial cells. In vivo, 1 week after administration of the hydrogel to a rabbit model of intrapericardial injury, superior efficacy was observed when compared to a commercial adhesion barrier, as histological and immunohistochemical examination revealed reduced adhesion formation and minimal immune infiltration of CD3+ lymphocytes and CD68+ macrophages, as well as NF-κß downregulation. We presented a novel nanostructured drug delivery hydrogel system with unique mechanical and biological properties that act synergistically to prevent cellular infiltration while providing local immunomodulation to protect the intrapericardial space after a surgical intervention.

[Percutaneous transluminal septal myocardial ablation in patients with obstructive septal hypertrophic myocardiopathy. Acute results and 3-year follow-up]. / Ablación miocárdica septal transluminal percutánea en pacientes con miocardiopatía hipertrófica septal obstructiva. Resultados agudos y seguimiento a 3 años.

OBJECTIVE: To report the acute and Long term results of percutaneous transluminal septal myocardial ablation (PTSMA), for the treatment of hypertrophic obstructive cardiomyopathy (HOCM). PTSMA has been considered as an alternative to surgical treatment in patients with HOCM and drug-refractory symptoms or unresponsive to the implantation of a pacemaker. Acute response is generally satisfactory, but long term results have not been sufficiently described. METHODS: We did PTSMA in eight patients, with functional class III-IV of NYHA, and with a transaortic gradient at rest > or = 40 mmHg. ALcohol was administered to seven of them, and small absorbant gelatin sponge particles in the other. Acute and long term clinical and echocardiographical follow-up was done. RESULTS: During the procedure the left ventricular outflow tract (LVOT) gradient at rest was reduced significantly and the systolic anterior motion of the mitral valve (SAM) also. Five remained on functional class I, and two in class II. One patient died one year later of sepsis unrelated to the cardiopathy. CONCLUSIONS: PTSMA is an excellent non surgical option to reduce the LOVT gradient and the consequent symptoms in this patients. Long term follow-up shows they remain in a good functional class and their evolution is free of cardiovascular complications.

Ablación miocárdica septal transluminal percutánea en pacientes con miocardiopatía hipertrófica septal obstructiva: resultados agudos y seguimiento a 3 años / Percutaneous transluminal septal myocardial ablation in patients with obstructive septal hypertrophic myocardiopathy: acute results and 3-year follow-up

OBJECTIVE: To report the acute and Long term results of percutaneous transluminal septal myocardial ablation (PTSMA), for the treatment of hypertrophic obstructive cardiomyopathy (HOCM). PTSMA has been considered as an alternative to surgical treatment in patients with HOCM and drug-refractory symptoms or unresponsive to the implantation of a pacemaker. Acute response is generally satisfactory, but long term results have not been sufficiently described. METHODS: We did PTSMA in eight patients, with functional class III-IV of NYHA, and with a transaortic gradient at rest > or = 40 mmHg. ALcohol was administered to seven of them, and small absorbant gelatin sponge particles in the other. Acute and long term clinical and echocardiographical follow-up was done. RESULTS: During the procedure the left ventricular outflow tract (LVOT) gradient at rest was reduced significantly and the systolic anterior motion of the mitral valve (SAM) also. Five remained on functional class I, and two in class II. One patient died one year later of sepsis unrelated to the cardiopathy. CONCLUSIONS: PTSMA is an excellent non surgical option to reduce the LOVT gradient and the consequent symptoms in this patients. Long term follow-up shows they remain in a good functional class and their evolution is free of cardiovascular complications.

Percutaneous septal ablation with absorbable gelatin sponge in hypertrophic obstructive cardiomyopathy.

The treatment of the hypertrophic obstructive cardiomyopathy (HOCM) by percutaneous transluminal septal myocardial ablation (PTSMA) with ethanol injection has greatly improved in the last years. This report describes the case of a patient with symptomatic drug-refractory HOCM who underwent an unsuccessful attempt to thrombose the septal artery by PTSMA with alcohol. It has therefore been decided to use small absorbant gelatin sponge (AGS) particles, obtaining immediate thrombotic occlusion of the artery and excellent hemodynamic results with immediate and permanent disappearance of the gradient. The patient progressed satisfactorily and displayed lower than average creatine kinase levels in comparison to the rest of our PTMSA patients. After 2 years of follow-up he still remains asymptomatic and without any gradient. These results suggest that PTSMA with AGS could be a valuable alternative treatment of HOCM.