Background: Hypertrophic cardiomyopathy (HCM) is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model in pediatric HCM to guide SCD prevention strategies. Methods: In an international multi-center observational cohort study, phenotype-positive patients with isolated HCM <18 years at diagnosis were eligible. The primary outcome variable was the time from diagnosis to a composite of SCD events at 5-year follow-up: SCD, resuscitated sudden cardiac arrest (SCA), and aborted SCD, i.e. appropriate shock following primary prevention ICD. Competing risk models with cause-specific hazard regression were used to identify and quantify clinical and genetic factors associated with SCD. The cause-specific regression model was implemented using boosting, and tuned with ten repeated four-fold cross-validations. The final model was fitted using all data with the tuned hyperparameter value that maximizes the c-statistic, and its performance was characterized using c-statistic for competing risk models. The final model was validated in an independent external cohort (SHaRe, n=285). Results: Overall, 572 patients met eligibility criteria with 2855 patient-years of follow-up. The 5-year cumulative proportion of SCD events was 9.1% (14 SCD, 25 resuscitated SCA, 14 aborted SCD). Risk predictors included age at diagnosis, documented non-sustained ventricular tachycardia, unexplained syncope, septal diameter z-score, LV posterior wall diameter z-score, LA diameter z-score, peak LV outflow tract (LVOT) gradient, and presence of a pathogenic variant. Unlike adults, LVOT gradient had an inverse association, and family history of SCD had no association with SCD. Clinical and clinical/genetic models were developed to predict 5-year freedom from SCD. Both models adequately discriminated patients with and without SCD events with a c-statistic of 0.75 and 0.76 respectively and demonstrated good agreement between predicted and observed events in the primary and validation cohorts (validation c-statistic 0.71 and 0.72 respectively). Conclusions: Our study provides a validated SCD risk prediction model with over 70% prediction accuracy and incorporates risk factors that are unique to pediatric HCM. An individualized risk prediction model has the potential to improve the application of clinical practice guidelines and shared decision-making for ICD insertion. Clinical Trial Registration: URL: https://clinicaltrials.gov Unique Identifier: NCT04036799
Purpose The prone position is required for posterior spinal fusion surgery and may be associated with cardiovascular changes, including a decrease in venous return and cardiac index. We report a case of a patient who developed cardiovascular collapse, increased central venous pressure (CVP), and massive bleeding during posterior spinal fusion surgery. A transesophageal echocardiography examination (TEE) documented a right ventricular outflow tract (RVOT) obstruction associated with the use of transverse bolsters. Clinical features We describe a case of a healthy 14-yr-old male with idiopathic scoliosis who developed severe intraoperative cardiovascular instability and massive bleeding. The surgery was suspended, and the patient was transferred to the intensive care unit. The patient subsequently underwent TEE in the supine and prone positions. The echocardiogram appeared normal in the supine position; however, in the prone position with transverse bolsters, we identified a significant decrease in the diameter of the RVOT that worsened with pressure applied against the thoracic spine. The central venous pressure increased from 10-24 mmHg simultaneously. We found appreciably less impact to the RVOT, RV size and flow, and CVP (10 to 14 mmHg) using longitudinal bolsters both with and without pressure to the back. This position was recommended for the patient's reoperation, which was uneventful. Conclusion A TEE confirmed a RVOT obstruction in the prone position that was associated, in this case, with the use of transverse bolsters. The RVOT obstruction was explained by the chest deformity, compliant chest cage, bolstering, and pressure applied to the patient's back by the surgeon. This positional RVOT obstruction may explain the increase in the CVP and the secondary massive bleeding during the first operation. The TEE was useful to diagnose the patient's condition and to guide his positioning for the second operation. RésuméObjectif La position ventrale est ne´cessaire pour les chirurgies du rachis par voie poste´rieure; cette position pourrait eˆtre associe´e a`des changements cardiovasculaires, notamment une re´duction du retour veineux et de l'index cardiaque. Nous rapportons le cas d'un patient qui
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