Right cervical aortic arch associated with anomaly of epiaortic vessels and haemodynamically significant obstruction between ascending and descending aorta. Extra-anatomic bypass graft between ascending and descending aorta and long-term follow-up.

Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop, descends, and crosses behind the oesophagus to continue as a left descending aorta. We present a case of right cervical aortic arch associated with anomaly of epiaortic vessels and aortic obstruction, surgically treated with extra-anatomic bypass graft between ascending and descending aorta.

Infradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience.

BACKGROUND AND OBJECTIVES: Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation. METHODS: Over a 10-year period, we observed seven adult patients (median age 29 years) with partial anomalous pulmonary venous connection. We classified our patients in two groups. Group I: isolated partial anomalous pulmonary venous connection from one pulmonary lobe to the inferior vena cava, three patients. Group II: partial anomalous pulmonary venous connection of the entire right lung to IVC, four patients. RESULTS: The mean term follow-up was 5.4 years. Patients in Group I have been managed conservatively, as they were asymptomatic, without a significant shunt. Patients in Group II were surgically corrected using long right intra-atrial baffles. After 6 months of follow-up, the first two cases were diagnosed with complete tunnel thrombosis and loss of right lung function. Oral anticoagulation failed to recanalize the tunnel. Considering this serious complication, the other two patients were empirically and preventively treated with anticoagulation after surgery, with good outcome on long-term follow-up. CONCLUSIONS: Conservative management should be considered for asymptomatic patients, without a significant shunt. Surgical treatment of infradiaphragmatic partial anomalous pulmonary venous connection of the entire right lung in inferior vena cava is challenging. Slow blood flow inside the long intra-atrial baffles inclines to thrombosis and occlusion, as we observed in two cases. Therefore, oral anticoagulation should be considered for long baffles with slow blood flow.

Congenital left ventricular aneurysm of interventricular septum: prenatal diagnosis and long-term management.

Congenital left ventricular aneurysm is a rare anomaly consisting of an extroflection from left ventricular walls with a wide connection. Most left ventricular aneurysms are asymptomatic and diagnosed as incidental finding, but can be associated with complications during follow-up. We present a case of congenital left ventricular aneurysm at the level of the interventricular septum with secondary remodelling of the left ventricle.

[Anomalous origin of the left coronary artery from the pulmonary artery treated with Takeuchi repair in early childhood. Long-term follow-up of two cases complicated by supravalvular pulmonary stenosis]. / Origine anomala della coronaria sinistra dall'arteria polmonare trattata con tecnica di Takeuchi nella prima infanzia. Follow-up a lungo termine di due casi clinici complicati da stenosi polmonare sopravalvolare.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but serious congenital heart defect. We report two cases of ALCAPA diagnosed in infancy, presenting with low body weight and severe symptoms of congestive heart failure. Both were repaired surgically using Takeuchi technique. Both cases have developed supravalvular pulmonary stenosis at long-term follow-up.

Clinical Characteristics and Long-Term Mortality Rate in Female Patients with Takotsubo Syndrome Compared with Female Patients with ST-Elevation Acute Myocardial Infarction: A Retrospective Study from a Single Center.

BACKGROUND: Takotsubo syndrome (TTS) is characterized by acute transient, stress-induced, left ventricular systolic dysfunction, generally presenting with apical ballooning. It can mimic an acute coronary syndrome, but with a milder increase in cardiac enzymes and without culprit coronary artery disease on angiography. Data on long-term follow-up and survival in patients with TTS, compared with patients with ST-elevation myocardial infarction (STEMI), are scarce. PURPOSE: To assess all-cause mortality rate and survival in a consecutive series of female patients with TTS compared with age- and sex-matched STEMI patients on long-term follow-up. METHODS AND RESULTS: We collected data of 65 TTS female patients (TTS group) with a mean age of 73.42 ± 11.35 years from 2001 to 2013. Collection of follow-up information was concluded for all patients in 2016. To compare the mortality and survival of TTS patients with those of the STEMI population, we used data from our STEMI Registry, a prospective registry of 7446 STEMI patients admitted from 2001 to 2013 to our cath-lab for primary percutaneous coronary intervention (p-PCI). From the registry, we selected 104 STEMI patients (STEMI group) comparable to our TTS group in terms of age (mean age of 72.33 ± 11.92 years) and sex. On follow-up examination after a median of 1000 days, the TTS group had a lower all-cause mortality rate than the STEMI group (7.69% versus 23.08%). This difference was statistically different between the two groups (log-rank test, p value = 0.03). CONCLUSIONS: In our study, TTS and STEMI patients displayed a statistically significant difference in long-term survival. Specifically, the TTS group had a lower mortality rate than the STEMI group. This seems to suggest that TTS and STEMI are two different clinical entities with two different clinical outcomes.

Diagnostic value of ischemia severity at myocardial perfusion imaging in elderly persons with suspected coronary disease.

AIMS: Myocardial perfusion and ischemia scores obtained from myocardial perfusion scintigraphy (MPS) have strong independent prognostic value in elderly individuals without known coronary artery disease (CAD). Herein we aimed to assess their independent diagnostic value and accuracy for CAD while considering different thresholds of myocardial ischemia. METHODS: We estimated the summed rest score (SRS), summed stress score (SSS) and summed difference score (SDS) in 322 elderly individuals (mean age 72 ±â€Š7 years, 68% men) who underwent coronary angiography following an MPS. Abnormal perfusion at stress was defined as an SSS greater than 3, and ischemia as an SDS of at least 2, and further categorized as mild (2-4), moderate (5-7) or severe (>7). Multivariate logistic regressions were used to establish the independent diagnostic value and accuracy of MPS parameters. RESULTS: CAD was diagnosed in 182 individuals (56%). In multivariate analysis accounting for clinical variables associated with CAD including the Framingham risk score, both SRS [odds ratio (OR) 1.09, 95% confidence interval (CI) 1.01-1.18, P = 0.03] and SSS (OR 1.10, 95% CI 1.04-1.16, P = 0.0006) and SDS (OR 1.12, 95% CI 1.04-1.21, P = 0.003) were independently associated with CAD. An SSS greater than 3 was also independently associated with CAD (OR 2.51, 95% CI 1.43-4.39, P = 0.0013), whereas an SDS of 2 or greater was not (OR 1.62, 95% CI 0.89-2.93, P = 0.12), but only when at least 5 (OR 2.31, 95% CI 1.32-4.03, P = 0.003). The probability of CAD was proportional to the amount of myocardial ischemia in those with an SSS greater than 3, and lower and comparable in those with an SSS of at least 3 or an SSS greater than 3 with SDS of 1 or less (P = 0.19). Increasing the threshold of myocardial ischemia determined a decrease in sensitivity and increase in specificity of MPS for both diagnosis and severity of CAD. CONCLUSION: We established the diagnostic value and accuracy of continuous scores and thresholds of abnormal myocardial perfusion and ischemia previously validated in prognostic studies. Their more widespread use could potentially improve the diagnostic yield of coronary angiography in elderly individuals with suspected CAD.