Improved Surgical Outcomes in Adults with Congenital Heart Disease and Decompensated Heart Failure: The Role of Perioperative Medical Optimization.

BACKGROUND Decompensated heart failure (HF) is recognized as a significant prognostic factor for unfavorable outcomes in both the general population and adults with congenital heart diseases (ACHD). Among ACHD patients, those with advanced heart failure may be candidates for heart transplantation. However, in ACHD patients requiring heart surgery, even with reduced ejection fraction, the administration of appropriate medications can result in improved circulatory parameters, functional class, and surgical outcomes. CASE REPORT We present 5 patients who exhibited indications for open-heart surgery, advanced heart failure (HF) accompanied by congestion, and significant physical activity limitations or symptoms at rest (NYHA class III-IV). Among the patients, 40% were male, with a mean age of 47.4 years (ranging from 32 to 62 years). Three patients displayed reduced systemic ventricular ejection fraction, while 4 patients experienced arrhythmia. Congenital heart diseases (CHD) observed in the patients included repaired double-outlet right ventricle, congenitally corrected transposition of the great arteries, repaired tetralogy of Fallot, partial anomalous right pulmonary venous return, and atrial septal defect. Comprehensive heart failure medications were administered, including an angiotensin receptor neprilysin inhibitor, levosimendan, beta-blockers, phosphocreatine, and diuretics. The preoperative period ranged from 8 days to 2 months. Notably, significant clinical and hemodynamic improvements were observed in all cases, and all open-heart surgeries were successfully completed. CONCLUSIONS Advanced and decompensated HF has a high impact on surgical outcomes. Preoperative care with prescribed medical management for ACHD patients is possible and provides good early and mid-term postoperative results.

A rare case of rebleeding brainstem cavernoma in a 5-month-old-girl.

Background: Brainstem cavernomas (BSCs) are relatively rare intracranial vascular lesions that, if left untreated, can be devastating to the patient. The lesions are associated with a myriad of symptoms, depending on their size and location. However, medullary lesions present acutely with cardiorespiratory dysfunction. We present the case of a 5-month-old child with a BSC. Case Description: A 5-month-old child presented for the 2nd time with sudden respiratory distress and excessive salivation. On the first presentation, brain magnetic resonance imaging (MRI) showed a 13 × 12 × 14 mm cavernoma at the pontomedullary junction. She was managed conservatively but presented 3 months later with tetraparesis, bulbar palsy, and severe respiratory distress. A repeat MRI showed enlargement of the cavernoma to 27 × 28 × 26 mm with hemorrhage in different stages. After hemodynamic stabilization, complete cavernoma resection was performed through the telovelar approach with neuromonitoring. Postoperatively, the child recovered motor function, but the bulbar syndrome persisted with hypersalivation. She was discharged on day 55 with a tracheostomy. Conclusion: BSCs are rare lesions that are associated with severe neurological deficits due to the compactness of important cranial nerve nuclei and other tracts in the brainstem. Early surgical excision and hematoma evacuation for superficially presenting lesions can be lifesaving. However, the risk of postoperative neurological deficits is still a major concern in these patients.