Project description:Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly which can be symptomatic or even asymptomatic. Most of patients with isolated UPAA have mild symptoms and it is difficult to be diagnosed, especially when abnormal findings of chest radiograph are the first presentation. It is often misdiagnosed and is not considered during differential diagnosis. To make a diagnosis of UPAA, various imaging modalities including chest radiograph, computed tomography (CT), and angiography are used. We report a 33-year-old woman in pregnancy presented recurrent hemoptysis whose CT was postponed due to her pregnancy. Although CT is a useful diagnostic tool, chest radiograph could be used instead in pregnancy suggesting UPAA with a lot of information.
Project description:IntroUnilateral pulmonary artery atresia (UPAA), while encountered frequently in the congenital cardiac anomaly cohort, is occasionally diagnosed in adulthood after typical symptoms of hemoptysis, pulmonary infection, or as an incidental finding on contrast CT scan. Due to its rarity, a brief discussion of UPAA and its treatment is warranted.Case reportA 35 year old male presented with three days of hemoptysis. After diagnosis of right UPAA, he underwent angioembolization of 6 large systemic collaterals supplying his right lung, followed by right pneumonectomy. He was discharged on post-operative day 3, and at follow up 6 weeks later was doing well with minimal residual incisional pain and excellent pulmonary reserve.ConclusionsUPAA presents classically with hemoptysis, but also with pneumonia, pulmonary hypertension, or incidentally. Management includes selective collateral embolization, pneumonectomy, or medical management directed towards decreasing pulmonary hypertension in patients unable to tolerate pneumonectomy due to comorbidities. Pneumonectomy in these patients is characterized by dense and hypervascular adhesions, with large volume blood loss expected during adhesiolysis, which can be decreased with pre-operative embolization. Outcomes are typically excellent in otherwise healthy patients.
Project description:This is a report on a 10-year-old child with anomalous origin of left coronary artery (LCA) from pulmonary artery (ALCAPA), severe pulmonary hypertension (PH), old myocardial infarction and poor intercoronary collateralization. It discusses the echocardiographic pitfalls in this particular setting and introduces a new echocardiographic view (posterior pulmonary cusp view) for visualization of the anomalous origin of LCA from the posterior pulmonary cusp (PC) in patients with ALCAPA from the PC of the pulmonary artery. We describe three echocardiographic pitfalls that can mislead the echocardiographer and two helpful hints that guide the clinician to the correct diagnosis. The survival of this child shows that limited size of left ventricular myocardial infarction and severe mitral regurgitation in early infancy can result in a life-saving pulmonary hypertension which preserves viability and function of left ventricle despite lack of intercoronary collateral arteries. After one year follow-up, she is doing well on medical treatment.
Project description:Background: Coronary collateral circulation protects cardiac tissues from myocardial infarction damage and decreases sudden cardiac death. So far, it is unclear how coronary collateralization varies by race-ethnicity groups and by sex.Methods: We assessed 868 patients with obstructive CAD. Patients were assessed for collateral grades based on Rentrop grading system, as well as other covariates. DNA samples were genotyped using the Affymetrix 6.0 genotyping array. To evaluate genetic contributions to collaterals, we performed admixture mapping using logistic regression with estimated local and global ancestry.Results: Overall, 53% of participants had collaterals. We found difference between sex and racial-ethnic groups. Men had higher rates of collaterals than women (P-value = 0.000175). White Hispanics/Latinos showed overall higher rates of collaterals than African Americans and non-Hispanic Whites (59%, 50% and 48%, respectively, P-value = 0.017), and especially higher rates in grade 1 and grade 3 collateralization than the other two populations (P-value = 0.0257). Admixture mapping showed Native American ancestry was associated with the presence of collaterals at a region on chromosome 17 (chr17:35,243,142-41,251,931, ? = 0.55, P-value = 0.000127). African ancestry also showed association with collaterals at a different region on chromosome 17 (chr17: 32,266,966-34,463,323, ? = 0.38, P-value = 0.00072).Conclusions: In our study, collateralization showed sex and racial-ethnic differences in obstructive CAD patients. We identified two regions on chromosome 17 that were likely to harbor genetic variations that influenced collateralization.
Project description:ObjectiveWe investigated whether and to what extent cystatin C was associated with angiographic coronary collateralization in patients with stable coronary artery disease and chronic total occlusion.MethodsSerum levels of cystatin C and high-sensitive C-reactive protein (hsCRP) and glomerular filtration rate (GFR) were determined in 866 patients with stable angina and angiographic total occlusion of at least one major coronary artery. The degree of collaterals supplying the distal aspect of a total occlusion from the contra-lateral vessel was graded as poor (Rentrop score of 0 or 1) or good coronary collateralization (Rentrop score of 2 or 3).ResultsIn total, serum cystatin C was higher in patients with poor collateralization than in those with good collateralization (1.08 ± 0.32 mg/L vs. 0.90 ± 0.34 mg/L, P < 0.001), and correlated inversely with Rentrop score (adjusted Spearmen's r = -0.145, P < 0.001). The prevalence of poor coronary collateralization increased stepwise with increasing cystatin C quartiles (P for trend < 0.001). After adjusting for age, gender, risk factors for coronary artery disease, GFR and hsCRP, serum cystatin C ? 0.97 mg/L remained independently associated with poor collateralization (OR 2.374, 95% CI 1.660 ~ 3.396, P < 0.001). The diagnostic value of cystatin C levels for detecting poor coronary collateralization persisted regardless of age, gender, presence or absence of diabetes, hypertension or renal dysfunction.ConclusionsSerum cystatin C reflects angiographic coronary collateralization in patients with stable coronary artery disease, and cystatin C ? 0.97 mg/L indicates a great risk of poor coronary collaterals.
Project description:Pulmonary agenesis, a rare congenital condition, is incompatible with life when present bilateral, while unilateral agenesis is usually detected in infancy or early childhood. Rare asymptomatic patients may reach adulthood undiagnosed, with signs mimicking common conditions presenting as radiopaque hemithorax with ipsilateral mediastinal shift. Here, we describe a case of a young lady, with history of consanguinity, who presented with complaints, suggestive of lower respiratory tract infection, and was investigated and diagnosed to be a case of right-side pulmonary agenesis with large ostium secondum atrial septal defect. Our present case emphasizes the importance of presence of pulmonary agenesis with cardiac congenital anomaly, remaining asymptomatic until adulthood, particularly in patients born of parents with consanguineous marriages.
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:A 2-month-old baby with ventricular septal defect and pulmonary atresia was found to have coronary-to-pulmonary artery collaterals. Cardiac computed tomography confirmed the coronary collaterals and showed the absence of other systemic to pulmonary artery collaterals. Although these collaterals do not cause coronary ischemia, it is important to delineate them by accurate imaging to plan the appropriate surgical strategy.