Subject(s)
Arteriovenous Fistula/therapy , Cardiac Catheterization/instrumentation , Coronary Vessel Anomalies/therapy , Coronary Vessels , Septal Occluder Device , Vena Cava, Inferior/abnormalities , Arteriovenous Fistula/diagnostic imaging , Child, Preschool , Coronary Angiography , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessels/diagnostic imaging , Echocardiography, Doppler, Color , Humans , Male , Prosthesis Design , Treatment Outcome , Vena Cava, Inferior/diagnostic imagingABSTRACT
We present a rare late manifestation of systemic venous collaterals in a 17-year-old female, 16 years after bidirectional cavopulmonary anastomosis, resulting in clinically unacceptable desaturation with progressive effort intolerance and cyanosis.
Subject(s)
Azygos Vein/abnormalities , Azygos Vein/diagnostic imaging , Cyanosis/diagnostic imaging , Fontan Procedure/adverse effects , Heart Defects, Congenital/diagnostic imaging , Adolescent , Collateral Circulation , Echocardiography , Female , Heart Defects, Congenital/surgery , Humans , Pulmonary Artery/surgery , Radiography , Tomography, X-Ray Computed , Vena Cava, Superior/surgeryABSTRACT
4 months male child presented with failure to thrive. On general examination child had normal O2 saturation with characteristic elfin facies. Further evaluation of the patient showed major manifestations of Williams syndrome in form of supravalvar aortic stenosis, branched pulmonary artery stenosis along with cardiomyopathy. Although the entity is known, this article shows comprehensive diagnostic workup with the aid of multimodality imaging techniques. The genetic diagnosis of Williams syndrome was confirmed using fluroscent in situ hybridisation techniques (FISH). In this patient most of the manifestations of elastin vasculopathy were noted in the form of involvement of ascending aorta, pulmonary arteries and myocardium. We also want to emphasis the importance of echocardiography in newborn patients with dysmorphic facies as Williams syndrome can be easily missed in neonatal period.
Subject(s)
Abnormalities, Multiple , Echocardiography, Doppler/methods , Imaging, Three-Dimensional/methods , Pulmonary Valve Stenosis/diagnosis , Tomography, X-Ray Computed/methods , Williams Syndrome/diagnosis , Diagnosis, Differential , Humans , Infant , MaleABSTRACT
A 26 year primigravida, 8 days after delivering a still born baby presented with gradually progressive dyspnea from New York Heart Association (NYHA) class II to class III over a period of 1 week. 2D-Echocardiography showed severely compromised left ventricular systolic function with evidence of thrombi in three different chambers of the heart. All the clots disappeared after an effective anticoagulation therapy without any embolic complications. The hypercoagulable state of the peripartum period and the severe biventricular dysfunction led to the formation of clots.
