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.

Right ventricular dysfunction and right ventricular-arterial uncoupling at admission increase the in-hospital mortality in patients with COVID-19 disease.

BACKGROUND: Coronavirus disease 2019 (COVID-19) frequently involves cardiovascular manifestations such as right ventricular (RV) dysfunction and alterations in pulmonary hemodynamics. We evaluated the application of the critical care ultrasonography ORACLE protocol to identify the most frequent alterations and their influence on adverse outcomes, especially those involving the RV (dilatation and dysfunction). METHODS: This cross-sectional study included 204 adult patients with confirmed COVID-19 admitted at three centers. Echocardiography and lung ultrasound images were acquired on admission using the ORACLE ultrasonography algorithm. RESULTS: Two-hundred and four consecutive patients were evaluated: 22 (11.9%) demonstrated a fractional shortening of < 35%; 33 (17.1%) a tricuspid annular plane systolic excursion (TAPSE) of < 17 mm; 26 (13.5%) a tricuspid peak systolic S wave tissue Doppler velocity of < 9.5 cm/sec; 69 (37.5%) a RV basal diameter of > 41 mm; 119 (58.3%) a pulmonary artery systolic pressure (PASP) of > 35 mm Hg; and 14 (11%) a TAPSE/PASP ratio of < .31. The in-hospital mortality rate was 37.6% (n = 71). Multiple logistic regression modeling showed that PASP > 35 mm Hg, RV FS of < 35%, TAPSE < 17 mm, RV S wave < 9.5, and TAPSE/PASP ratio < .31 mm/mm Hg were associated with this outcome. PASP and the TAPSE/PASP ratio had the lowest feasibility of being obtained among the investigators (62.2%). CONCLUSION: The presence of RV dysfunction, pulmonary hypertension, and alteration of the RV-arterial coupling conveys an increased risk of in-hospital mortality in patients presenting with COVID-19 upon admission; therefore, searching for these alterations should be routine. These parameters can be obtained quickly and safely with the ORACLE protocol.

Critical care ultrasonography during COVID-19 pandemic: The ORACLE protocol.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is characterized by severe lung involvement and hemodynamic alterations. Critical care ultrasonography is vital because it provides real time information for diagnosis and treatment. Suggested protocols for image acquisition and measurements have not yet been evaluated. METHODS: This cross-sectional study was conducted at two centers from 1 April 2020 to 30 May 2020 in adult patients with confirmed COVID-19 infection admitted to the critical care unit. Cardiac and pulmonary evaluations were performed using the ORACLE protocol, specifically designed for this study, to ensure a structured process of image acquisition and limit staff exposure to the infection. RESULTS: Eighty-two consecutively admitted patients were evaluated. Most of the patients were males, with a median age of 56 years, and the most frequent comorbidities were hypertension and type 2 diabetes, and 25% of the patients had severe acute respiratory distress syndrome. The most frequent ultrasonographic findings were elevated pulmonary artery systolic pressure (69.5%), E/e' ratio > 14 (29.3%), and right ventricular dilatation (28%) and dysfunction (26.8%). A high rate of fluid responsiveness (82.9%) was observed. The median score (19 points) on pulmonary ultrasound did not reveal any variation between the groups. Elevated pulmonary artery systolic pressure was associated with higher in-hospital mortality. CONCLUSION: The ORACLE protocol was a feasible, rapid, and safe bedside tool for hemodynamic and respiratory evaluation of patients with COVID-19. Further studies should be performed on the alteration in pulmonary hemodynamics and right ventricular function and its relationship with outcomes.

Thrombolytic treatment for acute prosthetic valve thrombosis: Is it better than surgery?

Prosthetic valve thrombosis (PVT) is a rare but often lethal complication of valve replacement. Despite advances in surgical techniques, surgical mortality remains high and emergent cardiac surgery is not widely available. Herein, we present the case of an acute PVT successfully treated with thrombolytic therapy. A 48-year-old female presented to the emergency department with acute onset of shortness of breath. Her medical history was remarkable for mitral valve replacement at the age of 42 years. Her symptoms began 4 h before, when she suddenly presented severe resting dyspnea. At arrival she showed pulmonary edema and hemodynamic instability. Echocardiography showed a prosthetic mitral valve stuck in a semi-closed position with a gradient of 39 mm Hg. Treatment was started with alteplase and the patient presented marked clinical improvement in subsequent hours. Recent evidence suggests that thrombolysis could be elected as the first choice of treatment in patients with left-side PVT because of the effectiveness, safety profile, availability, and low cost. This case illustrates the importance of prompt diagnosis and treatment and proposes thrombolytic therapy for PVT as an option at least as efficient as and far more accessible than surgery. .

Changes on craniofacial structures in children with growth-hormone-deficiency.

OBJECTIVE: To describe the growth of craniofacial structures in growth-hormone deficiency (GHD) children during growth-hormone therapy (GHT). METHODS: A cross-sectional sample of 46 subjects (n = 14 girls, 32 boys) aged 4-18 years was obtained. They were categorized into two paired groups: the reference group, for comparing the cephalograms, consisted in 23 healthy subjects, and the study group (23 patients) with GHD under GHT. Differences between groups were assessed by independent t-tests. RESULTS: The boys showed smaller measurements for all facial structures presenting significant differences in total mandibular length (Co-Pg p < 0.03), lower anterior facial height (ANS-Me p < 0.03) and total anterior facial height (N-Me p < 0.02) as well as retrognathic facial type. In girls the posterior cranial base length was shortened (S-Ba 29.14 ± 3.02 mm) and show a high mandibular plane angle (40 ± 5.50°) a wide relation anterior maxillo-mandibular (5.86 ± 1.57°) with a statistical difference (p < 0.05 and p < 0.04) compared with the reference group. CONCLUSIONS: We suggest considering the cephalometric morphology at the beginning of GHT.