RESUMEN
Sudden infant death syndrome (SIDS), the most common cause of infant death in developed countries, is attributed to diverse trigger factors. Malignant cardiac dysrhythmias are potentially treatable etiologies, and congenital long QT syndrome (LQTS) is the most common cardiac ionic channelopathy confronted. ß-Blockers or class Ib agents are the drugs of choice for the control of arrhythmias, and an implantable cardioverter defibrillator (ICD) should be considered for secondary prevention in survivors of lethal cardiac death. We report the case of a 4-day old neonate, later genetically confirmed as LQT type 3 (LQT3), who survived a pulseless torsades de pointes (TdP) attack and was successfully treated with propranolol, mexiletine, and ICD implantation.
RESUMEN
BACKGROUND: For type A aortic dissection (TAAD), antegrade cerebral perfusion (ACP) was proposed as a more physiological method than retrograde cerebral perfusion (RCP) for intra-operative brain protection, but it is still debatable whether antegrade cerebral perfusion (ACP) or retrograde cerebral perfusion (RCP) is related to the better clinical outcome. The present study was undertaken to compare the results in our patients receiving surgery for TAAD with ACP or RCP. The primary aim of this study was focused on the incidence of and the factors associated with surgical mortality, post-operative neurological outcomes and long-term survival. METHODS: From February 2001 to March 2019, there were 223 consecutive patients with TAAD treated surgically at our hospital. The median age at presentation was 56 years (range 29-88 years) and 70 patients (31.4%) over 65 years of age. There were 168 patients treated with RCP and 55 patients treated with ACP. The primary endpoints were surgical mortality and neurological outcome. Propensity score matching was used to compare the treatment results of surgeries with RCP or ACP. The long-term survival was also analyzed. RESULTS: The overall in-hospital mortality rate and the overall 30-day mortality rate were 15.6% and 14.3% respectively. For the patients without pre-operative shock (n = 184), the in-hospital mortality rate was 10.3% and the 30-day mortality rate was 8.7% and higher long-term survival rates (88.3% for 5 years, 86.5% for 10 years, 86.5% for 15 years) were documented for this patient group. There was no significant difference on the surgical mortality between the ACP group and the RCP group. In the entire cohort, there were 23 patients (10.3%) who suffered from post-operative neurological deficits (PND) and there were less PND for the patients with RCP than the patients with ACP (7.7% vs 18.1%, p = 0.027). After propensity score matching, there was still higher incidence of PND in the ACP group than in the RCP group but without statistical significance (18.5% vs 11.1%, p = 0.279). CONCLUSIONS: Aortic surgery carries high risk for the patients with TAAD and PND is not an unusual post-operative morbidity. In our series, pre-operative shock, pre-operative CPR, CRI, past history with CAD are related to higher surgical mortality. The younger patients (<65 years old) without pre-operative shock got better surgical outcome and long-term survival. RCP could provide acceptable cerebral protection during aortic surgery for the TAAD patients. Old age, pre-operative shock, CRI and past history of CAD are independent risk factors for long-term survival.