Spontaneous coronary artery rupture is a rare disorder that may develop early into a sudden death due to the abrupt evolution of the associated cardiac tamponade. In some cases the rupture is contained and a false aneurysm develops with slower evolution of clinical signs. The correct diagnosis of spontaneous coronary artery rupture deserves a high level of suspicion; frequently it may be missed because the time window of its evolution seems to be very short or signs of acute coronary syndrome sometimes can prevail, leading to delays in diagnosis or to misdiagnosis. We report the case of a patient presenting a giant pseudoaneurysm of the right coronary artery due to spontaneous coronary artery rupture without any underlying disease. Moreover we present a review of the few cases in the literature, offering a pathophysiological hypothesis linking the site of rupture and clinical presentation.
Physiologically corrected transposition of the great arteries (cTGA), defined by discordant atrioventricular and ventriculoarterial connections, is an uncommon congenital cardiac malformation. It rarely exists without associated cardiac anomalies, the most common of which are ventricular septal defect, pulmonary outflow obstruction, tricuspid valve (systemic) deformity, and rhythm disturbances. Conversely, hypoplasia of the systemic ventricle and systemic inflow or outflow obstructions have seldom been reported, although their recognition may significantly influence surgical repair and the patient's prognosis. We report a case of cTGA with complete heart block, moderate hypoplasia of the systemic ventricle, and severe aortic coarctation that was echocardiographically diagnosed in utero at 30 weeks' gestation because of fetal growth retardation and persistent fetal bradycardia. After delivery the patient underwent epimyocardial pacemaker implantation and aortic coarctation repair at 2 weeks of age. Unfortunately, the patient died on the seventh postoperative day because of systemic ventricular hypertrophy. Although it is well known that fetal echocardiography may reliably diagnose uncommon congenital cardiac malformations, to the best of our knowledge, this paper represents the first reported case of antenatal diagnosis of this complex anomaly.
Background and Aim of the Study
To report early clinical outcomes of the frozen elephant trunk (FET) technique for the treatment of complex aortic diseases after transition from conventional elephant trunk.
Methods
A single‐center, retrospective study of patients who underwent hybrid aortic arch and FET repair for aortic arch and/or proximal descending aortic aneurysms, acute and chronic Stanford type A aortic dissection with arch and/or proximal descending involvement, Stanford type B acute and chronic aortic dissections with retrograde aortic arch involvement.
Results
Between December 2017 and May 2020, 70 consecutive patients (62.7 ± 10.6 years, 59 male) were treated: 41 (58.6%) for emergent conditions and 29 (41.4%) for elective. Technical success was 100%. In‐hospital mortality was 14.2% (n = 12, 17.1% emergent vs. 10.3% elective, P = NS); 2 (2.9%) major strokes; 1 (1.4%) spinal cord injury. Mean follow‐up was 12.5 months (interquartile range, 3.7–22.3). Overall survival at 3, 6, 12, and 24 months was 90% (95% confidence interval [CI], 83.2—97.3), 85.6% (95% CI, 77.7–94.3), 79.1% (95% CI, 69.9–89.5), 75.6% (95% CI, 65.8–86.9) and 73.5% (95% CI, 63.3–85.3). There were no aortic re‐interventions and no distal stent graft‐induced new entry (dSINE); 5 patients with residual type B dissection underwent TEVAR completion.
Conclusions
In a real‐world setting, FET with Thoraflex Hybrid demonstrated feasibility and good clinical outcomes, even in emergent setting. Our implant technique optimize cerebral perfusion reporting good results in terms of neurological complications. Techniques to perfect the procedure and to reduce remaining risks, and consensus on considerations such as standardized cerebral protection need to be reported.
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