Clinical Characteristics and Outcomes in Adults With Moderate-to-Severe Complexity Congenital Heart Disease Undergoing Palliation or Surgical Repair.

Background: Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood. Methods: We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up. Results: Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80-181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (P = 0.03). Conclusions: Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient's suitability for surgical management and to achieve the best clinical outcome for this population.

Contexte: Grâce aux avancées réalisées en matière de techniques diagnostiques et thérapeutiques, la survie des patients atteints d'une cardiopathie congénitale s'est considérablement améliorée. Cependant, en ce qui concerne les personnes atteintes d'une cardiopathie congénitale non corrigée présentant une complexité modérée ou extrême, les données portant sur les facteurs de risque prédictifs ainsi que sur les résultats chirurgicaux sont rares. Notre objectif était de décrire les résultats chirurgicaux ainsi que les facteurs prédictifs des résultats obtenus en milieu hospitalier chez les patients adultes atteints d'une cardiopathie congénitale présentant une complexité modérée ou extrême qui n'a pas été corrigée pendant l'enfance. Méthodologie: Nous avons mené une étude de cohorte rétrospective comprenant 49 patients adultes atteints d'une cardiopathie congénitale modérée ou complexe qui ont reçu leurs traitements dans un seul centre médical. Les variables cliniques et échocardiographiques ont été obtenues au moment de l'admission, après les interventions chirurgicales et pendant la période de suivi. Résultats: Les patients étaient en majorité des femmes (66 %). La fraction d'éjection du ventricule gauche ainsi que la fraction de raccourcissement de la voie d'éjection ventriculaire droite sont demeurées dans les limites de la normale. La pression systolique médiane de l'artère pulmonaire a été de 37 mmHg (27-55 mmHg). Le temps médian écoulé pour la circulation extracorporelle a été de 118 minutes (80-181 minutes) et pour le clampage de la crosse aortique, de 76 minutes (49-121 minutes). Le bloc auriculo-ventriculaire postopératoire complet a été la complication la plus fréquente (12,2 %). Le taux de survie en milieu hospitalier a été de 87,7 %. Le développement du syndrome du faible débit cardiaque accompagné d'une insuffisance prédominante du ventricule droit durant la période postopératoire a constitué le principal facteur prédictif de décès à l'hôpital (p = 0,03). Conclusion: Il est difficile de traiter les adultes qui présentent une cardiopathie congénitale modérée ou sévère non corrigée. Il est essentiel que les évaluations cliniques, fonctionnelles et par imagerie soient réalisées de façon adéquate pour déterminer si une prise en charge chirurgicale convient aux patients et pour garantir les meilleurs résultats cliniques chez ces derniers.

[Treatment of cardiogenic shock due to right ventricular involvement]. / Tratamiento del choque cardiogénico con compromiso ventricular derecho.

The right ventricle is susceptible to changes in preload, afterload, and contractility. The answer is its dilation with dysfunction/acute failure; filling is limited to the left ventricle and cardiac output. Systemic venous congestion is retrograde to the right heart, it is involved in the genesis of cardiogenic shock due to right ventricle involvement. This form of shock is less well known than that which occurs due to left ventricular failure, therefore, treatment may differ. Once the primary treatment has been carried out, since no response is obtained, supportive treatment aimed at ventricular pathophysiology will be the next option. It is suggested to evaluate the preload for the reasoned indication of liquids, diuretics or even ultrafiltration. Restore or maintain heart rate and sinus rhythm, treat symptomatic bradycardia, arrhythmias that make patients unstable, use of temporary pacing or cardioversion procedures. Improving contractility and vasomotility, using vasopressors and inotropes, alone or in combination, the objective will be to improve right coronary perfusion pressure. Balance the effect of drugs and maneuvers on preload and/or afterload, such as mechanical ventilation, atrial septostomy and pulmonary vasodilators. And the increasing utility of mechanical support of the circulation that has become a useful tool to preserve/restore right heart function.

El ventrículo derecho es susceptible a cambios en la precarga, poscarga y la contractilidad y la respuesta fisiopatológica es la dilatación con disfunción/falla aguda lo que limita el llenado del ventrículo izquierdo y el gasto cardiaco. La congestión venosa sistémica, está implicada en la génesis del choque cardiogénico con compromiso del ventrículo derecho. Esta forma de choque es menos conocida que la que sucede por falla ventricular izquierda, por ende, el tratamiento puede diferir. La primera línea de tratamiento son las medidas de soporte y en caso de no funcionar, el tratamiento dirigido a la fisiopatología ventricular será la siguiente opción. Se sugiere evaluar la precarga para la indicación razonada de líquidos, diuréticos o la ultrafiltración. Restaurar o mantener la frecuencia cardiaca y el ritmo sinusal, tratar la bradicardia sintomática, las arritmias que inestabilizan a los pacientes, el uso de marcapaso temporal o procedimientos de cardioversión. Mejorar la contractilidad y vasomotilidad a través del uso de vasopresores e inotrópicos, solos o combinados, el objetivo será mejorar la presión de perfusión coronaria derecha. Balancear el efecto de fármacos y maniobras en la precarga y/o poscarga, como la ventilación mecánica, septostomía atrial y vasodilatadores pulmonares. Y la creciente utilidad del soporte mecánico de la circulación que se ha convertido en una herramienta útil para preservar/restaurar la función cardiaca derecha.

[Anatomy-physiology considerations for cardiogenic shock with right ventricular involvement].

Since the discovery of right ventricular infarction, interest in the characteristics of the right ventricle has been increasing. Right ventricular function is now known to be a predictor of mortality in different settings. The right ventricle is a low-pressure, high-compliance, high-volume chamber. To carry out its normal function, it is coupled to the pulmonary circulation and the left ventricle. In the face of acute changes in pressure, volume overload and ischemia, it dilates to adapt to its new load. Its manifestation may be ventricular dysfunction and/or failure that will progress to cardiogenic shock due to right ventricular involvement. Various entities may be the cause of acute dysfunction: right ventricular infarction (alterations in contractility due to ischemia) and high-risk pulmonary thromboembolism (increased afterload). Both share a similar ventricular pathophysiology and high mortality without treatment. Understanding anatomy and physiology, dysfunction and acute ventricular failure are important to define a convenient diagnosis and treatment oriented towards pathophysiology. In this first part, the anatomy and physiology, acute right ventricular dysfunction/failure and cardiogenic shock are taken into consideration, from the perspective of these two entities. In another paper, treatment aimed at cardiogenic shock due to right ventricular involvement will be reviewed.

Desde el conocimiento del infarto del ventrículo derecho, el interés por las características del ventrículo derecho ha sido cada vez mayor. Ahora se sabe que la función ventricular derecha es un predictor de mortalidad en diferentes contextos. El ventrículo derecho es una cavidad de baja presión, alta compliancia y alto volumen. Para llevar a cabo su función normal se encuentra acoplado a la circulación pulmonar y al ventrículo izquierdo. Ante alteraciones agudas de sobrecarga de presión, volumen e isquemia, se dilata para adaptarse a su nueva carga. Su manifestación puede ser disfunción o falla ventricular que progresará a choque cardiogénico por involucro del ventrículo derecho. Diversas entidades pueden ser la causa de la disfunción aguda: el infarto del ventrículo derecho (alteraciones de la contractilidad por isquemia) y la tromboembolia pulmonar de alto riesgo (aumento de la poscarga). Ambas comparten una fisiopatología ventricular similar y alta mortalidad sin tratamiento. Entender la anatomía fisiológica, la disfunción y la falla ventricular aguda es importante para definir un diagnóstico oportuno y un tratamiento orientado a la fisiopatología. En esta primera parte se toma en consideración la anatomía fisiológica y la disfunción/falla aguda ventricular derecha y su desenlace en el choque cardiogénico, desde la perspectiva de estas dos entidades. En otro trabajo se revisará el tratamiento orientado al choque cardiogénico por involucro ventricular derecho.

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.