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Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

ABSTRACT: An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called "scimitar vein" without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.

SUBMITTER: O'Byrne ML

PROVIDER: S-EPMC6112172 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

O'Byrne Michael L ML Cross Russell R RR Martin Gerard R GR

Cardiology in the young 20170829 2

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompressio ...[more]

PMID: 28847332

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Project description:Iliac vein compression syndrome (IVCS) is an important cause of deep vein thrombosis, but the incidence of IVCS is still unclear. The purpose of this prospective study was to determine the incidence of IVCS in an asymptomatic patient population and to evaluate the risk factors in patients with and without IVCS.From October 2011 to November 2012, a total of 500 patients (228 women and 272 men; mean age of 55.4 ± 14.7 years) with no vascular-related symptoms were enrolled in this study. Computed tomography was performed to evaluate all patients. The degree of venous compression was calculated as the diameter of the common iliac vein at the site of maximal compression divided by the mean diameter of the uncompressed proximal and caudal left common iliac vein (LCIV). We compared the stenosis rate of the common iliac vein in women and men according to age and followed up patients to evaluate outcomes.The mean compression degree of the LCIV was 16% (4%, 36%); 37.8% of patients had a compression degree ?25% and 9.8% had a compression degree ?50%. There was a significant difference between men and women in the LCIV compression degree (9% [3%, 30%] vs. 24% [8%, 42%]; U = 4.66, P< 0.01). In addition, the LCIV compression degree among younger women (?40 years) was significantly different compared with that in older women (>40 years) (42% [31%, 50%] vs. 19% [5%, 39%]; U = 5.14, P< 0.001). Follow-up was completed in 367 patients with a mean follow-up of 39.5 months (range, 6-56 months). The incidence of IVCS in the follow-up period was 1.6%. Stenosis rate and the diameter of the site of maximal compression correlated with the incidence of IVCS. Multivariable Cox regression analysis showed that the stenosis rate was an independent risk factor of IVCS (Wald ?2 = 8.84, hazard ratio = 1.13, P< 0.001).The incidence of IVCS was low and correlated with the stenosis rate of iliac vein. Preventative therapy may be warranted for common iliac vein compression in patients at an increased risk of venous thromboembolism, especially patients with a higher iliac vein compression degree.

Project description:Anomalous right coronary artery from pulmonary artery (ARCAPA) is one of four known anomalies of the coronary arteries originating from the pulmonary artery. ARCAPA is a rare congenital anomaly that is associated with possible long-term complications including myocardial ischemia and left ventricular dysfunction. Clinical presentation can vary from asymptomatic murmur to angina, dyspnea, fatigue, congestive heart failure, myocardial infarction and even cardiac arrest. We present a case of a 52-year-old male who presented to the hospital for intermittent chest pain. The patient was vitally stable and examination was unremarkable. Upon workup, electrocardiogram (EKG) on admission showed no acute ischemic changes and troponin were <0.03 ng/mL. The patient was evaluated by cardiology and had a dobutamine stress echocardiogram (ECHO) for ischemic evaluation. During the recovery phase of dobutamine stress ECHO, the patient reported having 10/10 chest pain with a drop in blood pressure to 90 mmHg systolic. He was subsequently given sublingual nitroglycerin and fluids. Pain did resolve and blood pressure improved after treatment. No wall motion abnormalities were noted on ECHO, but there were inferior ST depressions on EKG during the stress portion of the test. The patient had repeated blood work after the stress test which showed an elevated troponin level of 0.08 ng/mL. The patient was taken to the catheterization lab for diagnostic coronary angiogram. Cardiac catheterization showed a right coronary artery (RCA) arising from the pulmonary artery and diagnosis of isolated ARCAPA was made because of his intermittent chest pain. No intervention was performed as no significant lesions were found. The patient was started on medical therapy including aspirin, beta blocker and statin. The patient did not want to pursue surgery at that point and he did not want any further intervention. He was discharged home with instructions to follow up with cardiology in an outpatient setting to be referred for surgical repair. The purpose of writing this case is to increase awareness among physicians to be able to recognize possible anomalous coronary artery origins during cardiac catheterization and to understand the risk of long-term complications of ARCAPA and need for surgical repair.

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Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

Publications

Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

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