Project description:Abernethy malformation, or congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Despite its rarity, it is increasingly being reported, and therefore, it is important to diagnose given the potential adverse clinical consequences if left untreated. It has a spectrum of presentations, ranging from complete lack of symptoms, to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We herein describe the case and echocardiographic, computed tomography, and magnetic resonance imaging findings of a transgender individual, with this anomaly detected incidentally during adulthood.
Project description:BackgroundAlthough rare, external compression of the left main coronary artery (LMCA) by a pulmonary arterial aneurysm (PAA) as a consequence of pulmonary arterial hypertension causing stable angina pectoris is well described. However, acute myocardial infarction is extremely rare, particularly with a full array of electrocardiographic, biochemical, and echocardiographic features, as in this scenario.CaseIn this case, a 62-year-old man with a past history of severe fibrotic lung disease was hospitalised with chest pain. The patient had dynamic anterolateral ischaemic changes on electrocardiography and serially elevated high-sensitivity troponin I. Transthoracic echocardiography revealed impaired left ventricular ejection fraction with anterolateral hypokinesis. Coronary angiography with intracoronary imaging revealed external compression of the LMCA. Computer tomography (CT) scans confirmed new PAA, compared to previous scans. The patient was successfully treated by percutaneous coronary stent implantation.ConclusionProgressive dilatation of the pulmonary artery due to pulmonary arterial hypertension can result in acute MI secondary to external compression of the LMCA. Clinicians should be mindful of acute coronary syndromes in patients with long-standing pulmonary hypertension presenting with chest pain.
Project description:Acute myocardial infarction (AMI) caused by severe stenosis of left main coronary artery (LMCA) presenting with cardiogenic shock and pulmonary edema during noncardiac surgery is uncommon, but a catastrophic event. A 77-year-old male with cholangiocarcinoma underwent hepatectomy. During the surgery, he presented with cardiogenic shock, which did not respond to infusion administration or vasopressor. A transesophageal echocardiogram revealed anterior, septal, and lateral severe hypokinesia and impaired left ventricular function. Emergent coronary angiogram showed severe stenosis of LMCA. The patient underwent primary percutaneous coronary intervention (PCI) under the support of intra-aortic balloon pump, followed by extracorporeal membrane oxygenation. The chest roentgenogram showed pulmonary edema. Two days after PCI, he successfully underwent hepatectomy and bile duct resection. Early identification of the cause of hemodynamic instability during noncardiac surgery and invasive strategy are important for minimizing the myocardial injury and improving clinical outcomes in AMI of LMCA.
Project description:AimsThe aim of this study is to evaluate the contemporary use of a pulmonary artery catheter (PAC) in acute myocardial infarction-cardiogenic shock (AMI-CS).Methods and resultsA retrospective cohort of AMI-CS admissions using the National Inpatient Sample (2000-2014) was identified. Admissions with concomitant cardiac surgery or non-AMI aetiology for cardiogenic shock were excluded. The outcomes of interest were in-hospital mortality, resource utilization, and temporal trends in cohorts with and without PAC use. In the non-PAC cohort, the use and outcomes of right heart catheterization was evaluated. Multivariable regression and propensity matching was used to adjust for confounding. During 2000-2014, 364 001 admissions with AMI-CS were included. PAC was used in 8.1% with a 75% decrease during over the study period (13.9% to 5.4%). Greater proportion of admissions to urban teaching hospitals received PACs (9.5%) compared with urban non-teaching (7.1%) and rural hospitals (5.4%); P < 0.001. Younger age, male sex, white race, higher comorbidity, noncardiac organ failure, use of mechanical circulatory support, and noncardiac support were independent predictors of PAC use. The PAC cohort had higher in-hospital mortality (adjusted odds ratio 1.07 [95% confidence interval 1.04-1.10]), longer length of stay (10.9 ± 10.9 vs. 8.2 ± 9.3 days), higher hospitalization costs ($128 247 ± 138 181 vs. $96 509 ± 116 060), and lesser discharges to home (36.3% vs. 46.4%) (all P < 0.001). In 6200 propensity-matched pairs, in-hospital mortality was comparable between the two cohorts (odds ratio 1.01 [95% confidence interval 0.94-1.08]). Right heart catheterization was used in 12.5% of non-PAC admissions and was a marker of greater severity but did not indicate worse outcomes.ConclusionsIn AMI-CS, there was a 75% decrease in PAC use between 2000 and 2014. Admissions receiving a PAC were a higher risk cohort with worse clinical outcomes.
Project description:Left main coronary artery (LMCA) disease due to external compression by a dilated main pulmonary artery (MPA) is an uncommon clinical entity. Here, we describe a 52-year-old woman with pulmonary arterial hypertension (PAH) and anteroseptal old myocardial infarction (OMI). The cause of the OMI was external compression of the LMCA by the dilated MPA and aneurysm of the left coronary sinus of Valsalva. The patient's sister (aged 56 years) had also been diagnosed with PAH and both women had a novel heterozygous splicing mutation, IVS2-2A?>?G (c.374-2A?>?G in NM_001456), in the filamin A ( FLNA) gene. To our knowledge, this is the first report of HPAH which is likely to be due to FLNA mutation and compression of the LMCA between a dilated MPA and aneurysm of the left coronary sinus of Valsalva.
Project description:The usual sources of pulmonary blood flow in pulmonary atresia (PA) with(VSD) are patent ductus arteriosus and aortopulmonary collaterals. However, rarely fistulous collaterals may also arise from the coronary arteries which usually open into the main pulmonary trunk or branch pulmonary arteries. In such cases, selective coronary angiogram may be required for the demonstration of pulmonary arterial anatomy. A case of PA with VSD with failure to demonstrate pulmonary arteries on routine catheterization study (ventricular, aortic root, and descending aortic angiograms) is being presented here. A coronary artery-to-pulmonary artery fistula was suspected in view of dilated left main coronary artery, and pulmonary arteries were well demonstrated with selective coronary angiogram.
Project description:Portosystemic shunts are rare congenital malformations that come to attention due to various hepatic and extrahepatic manifestations. Management of this malformation is dictated by the presence and adequacy of intrahepatic portal radicals. Staged occlusion of the shunt is recommended in patients with severely hypoplastic portal radicals. We describe a novel transcatheter technique that we improvised for staged occlusion of Abernethy malformation in a 2-year-old girl.