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BACKGROUND: Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events. CASE PRESENTATION: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg. CONCLUSION: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

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BACKGROUND: Hypertension (HTN) is the most significant modifiable risk factor for cardiovascular disease (CVD) and overall mortality. HTN is defined as a systolic blood pressure of ≥140 mmHg and/or a diastolic blood pressure of ≥ 90 mmHg. Generally, arrhythmias are characterized by a disruption of the heart's regular rhythm. They are strongly associated with increased risks of CVDs and sudden death. The most common arrhythmia worldwide is atrial fibrillation (AF). HTN, diabetes mellitus (DM), and coronary artery disease (CAD) are major risk factors for arrhythmias. OBJECTIVE: We aimed to identify the postoperative effects and risk factors of HTN and cardiac arrhythmia in patients who underwent cardiac surgery at King Abdulaziz University Hospital (KAUH) from 2015 to 2022. METHODS: A retrospective record review was conducted by collecting data from KAUH electronic medical records. A total of 402 patients participated in this study. This study includes all hypertensive and arrhythmic patients who underwent cardiac surgeries. RESULTS: Of the 402 patients studied, 209 had pre-operative HTN, and 47 had preoperative AF. Developing post-operative arrhythmia was found to significantly increase perioperative morbidity and mortality (p < 0.001). Risk factors for HTN and arrhythmia included increased age, higher BMI, and DM. CONCLUSION: The findings of this study suggest an association between preoperative HTN and AF and elevated rates of postoperative morbidity and mortality. AF emerged as the predominant arrhythmia type. It is advisable to optimize patients' health status prior to surgical procedures. Moreover, further research is recommended in this field to deepen our understanding of the perioperative implications of HTN and arrhythmias.

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OBJECTIVE: Many studies still dispute the identification of independent risk factors that influence outcome after neonatal cardiac surgery. We present our study to announce the contemporary outcomes and risk profile of neonatal cardiac surgery at our institute. METHODS: We designed a retrospective study of neonatal patients who underwent surgery for congenital heart diseases between June 2011 and April 2020. Demographic, operative, and postoperative data were collected from medical records and surgical databases. The primary outcome was the operative mortality (in-hospital death) and secondary outcomes included hospital length of stay, intensive care unit stay, duration of mechanical ventilation. RESULTS: In total, 1155 cardiac surgeries in children were identified; of these, 136 (11.8%) were performed in neonates. Arterial switch operations (48 cases) were the most frequent procedures. Postoperatively, 11 (8.1%) patients required extracorporeal membrane oxygenation, and 4 (2.9%) patients had complete heart block. Postoperative in-hospital mortality was 11%. The median postoperative duration of mechanical ventilation, intensive care unit stay, and hospital length of stay were 6, 18, and 24 days, respectively. CONCLUSION: The early outcomes of neonatal cardiac surgery are encouraging. The requirement of postoperative extracorporeal membrane oxygenation support, postoperative intracranial hemorrhage, and acute kidney were identified as independent risk factors of mortality following surgery for congenital heart defects in neonates.

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Ascending thoracic aortic aneurysms are rare in childhood and typically are seen in the setting of connective tissue defect syndromes. These aneurysms may lead to rupture, dissection, or valvular insufficiency, so root replacement is recommended. Here, we present a 17-month-old girl who presented with fever, cough, and pericardial effusion. Initially, we suspected this could be a COVID-19 case, so a nasopharyngeal swap was performed. An ascending aorta aneurysm involving the aortic arch was confirmed by echo, and urgent ascending aorta and arch replacement were done by utilizing the descending aorta as a new arch. The final diagnosis came with cutis laxa syndrome. In similar cases, good outcomes can be achieved with accurate diagnosis and appropriate surgical management.

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BACKGROUND: Re-exploration of bleeding after cardiac surgery is associated with significant morbidity and mortality. Perioperative blood loss and rate of re-exploration are variable among centers and surgeons. OBJECTIVE: To present our experience of low rate of re-exploration based on adopting checklist for hemostasis and algorithm for management. METHODS: Retrospective analysis of medical records was conducted for 565 adult patients who underwent surgical treatment of congenital and acquired heart disease and were complicated by postoperative bleeding from Feb 2006 to May 2019. Demographics of patients, operative characteristics, perioperative risk factors, blood loss, requirements of blood transfusion, morbidity and mortality were recorded. Logistic regression was used to identify predictors of re-exploration and determinants of adverse outcome. RESULTS: Thirteen patients (1.14%) were reexplored for bleeding. An identifiable source of bleeding was found in 11 (84.6%) patients. Risk factors for re-exploration were high body mass index, high Euro SCORE, operative priority (urgent/emergent), elevated serum creatinine and low platelets count. Re-exploration was significantly associated with increased requirements of blood transfusion, adverse effects on cardiorespiratory state (low ejection fraction, increased s. lactate, and prolonged period of mechanical ventilation), longer intensive care unit stay, hospital stay, increased incidence of SWI, and higher mortality (15.4% versus 2.53% for non-reexplored patients). We managed 285 patients with severe or massive bleeding conservatively by hemostatic agents according to our protocol with no added risk of morbidity or mortality. CONCLUSION: Low rate of re-exploration for bleeding can be achieved by strict preoperative preparation, intraoperative checklist for hemostasis implemented by senior surgeons and adopting an algorithm for management.

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