An impressive image of unilateral pulmonary artery agenesis associated with coronary collateralization in an adult.
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ABSTRACT:
SUBMITTER: Alsancak Y
PROVIDER: S-EPMC7724390 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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.
| S-EPMC6232625 | biostudies-literature
Project description:Background Unilateral pulmonary artery agenesis (UPAA) and Kommerell's diverticulum (KD) are two rare embryologically unrelated congenital vascular malformations rarely diagnosed in children. This is the first report of our knowledge of the unique combination for a child as patients are at a high risk of pulmonary hypertension and rupture of the diverticulum. Our aim is to present the case of a pediatric patient with UPAA and KD with the short literature review and to highlight the importance of early diagnostics of rare congenital vascular malformations. Case report A 1-year-old girl presented to the emergency department with prolonged cough and variable wheezing. A hypoplastic left lung was suspected in the radiographic image of the chest. A transthoracic echocardiogram revealed absence of the left pulmonary artery and right arch of aorta and anomaly of subclavian arteries was suspected. The diagnosis was confirmed by computed tomography scans of the chest that demonstrated elongation of the aorta and an aberrant right subclavian artery with KD, as well as absence of the left pulmonary artery. The patient is being followed up for the development of pulmonary hypertension and compression of vascular structures to the airways as well as any indications for surgical intervention because of the KD. Conclusions UPAA and KD are two very rare congenital vascular anomalies usually diagnosed in adults. A high risk of pulmonary hypertension and rupture of diverticulum is noted for adult patients. This case provides us with an exclusive possibility to follow up a patient with an extremely rare combination of the two vascular anomalies with insufficiently known future complications and outcomes.
| S-EPMC10424440 | biostudies-literature
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.
| S-EPMC6297891 | biostudies-other
Project description:This is a case report of a 53-year-old woman who presented to our hospital in 2011 with an intermittent cough and dyspnea for 5 years. The chest X-ray showed a prominent left hilum and a smaller right lung. Computed tomography (CT) of the chest confirmed the absence of the right pulmonary artery (PA) and the right cardiac catheterization showed a mean PA pressure of 34 mmHg. Concomitant asthma and unilateral absence of pulmonary artery (UAPA) were diagnosed. In the following years, her functional class remained stable under medications including low-dose sildenafil and spironolactone. In 2020, she developed mild intermittent chest tightness and the coronary angiography showed a fistula between the proximal left circumflex coronary artery and right pulmonary circulation. She declined further intervention for her coronary-pulmonary artery fistula (CPAF) and her symptoms improved spontaneously. To our knowledge, only 16 similar cases with combined UAPA and CPAF in adults have been reported in the literature, of which, pulmonary hypertension was documented in nine patients (56.3%).
| S-EPMC9679234 | biostudies-literature
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.
| S-EPMC4310958 | biostudies-literature
Project description: Not available
| S-EPMC10784611 | biostudies-literature
Project description: Not available
| S-EPMC10954674 | biostudies-literature
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.
| S-EPMC5634541 | biostudies-literature
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.
| S-EPMC4581619 | biostudies-literature
Project description: Not available
| S-EPMC6791095 | biostudies-literature