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An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.
This article was published in the following journal.
Name: Echocardiography (Mount Kisco, N.Y.)
Eisenmenger syndrome refers to the elevation of pulmonary arterial pressure to the systemic level caused by an increased pulmonary vascular resistance with right-to-left shunt through an intracardiac ...
Compared to primary pulmonary hypertension (PPH), the right ventricular (RV) contractile function is preserved for a long time in patients with Eisenmenger syndrome and is likely the most important de...
Eisenmenger syndrome is a rare sequela of uncorrected congenital heart disease complicated by pulmonary hypertension, from which reversal of the pathologic left-to-right cardiovascular shunt and cyano...
Wilkie's syndrome (WS), also known as superior mesenteric artery syndrome, is a rare clinical entity caused by compression of the horizontal (third) part of the duodenum between the superior mesenteri...
Platypnoea-orthodeoxia syndrome (POS) is a rare clinical entity. It is characterized by position-dependent dyspnoea and oxygen desaturation in the upright position with orthodeoxia resolving in the su...
Long-term study to evaluate if macitentan is safe, tolerable and efficient enough to be used for treatment of Eisenmenger syndrome.
This is a historical cohort study with retrospective collection of data comprising all Nordic patient's diagnosed with Eisenmengers syndrome in the period 1977 through 2011. The goal is to...
Eisenmenger Quality Enhancement Research Initiative (QuERI) is a multi-center, observational, US-based longitudinal program, with enrollment of consecutive Eisenmenger patients who meet en...
The purpose of this study was to evaluate the acute effects of oxygen supplementation during a submaximal exercise test in patients with Eisenmenger's Syndrome.
The purpose of this study is to investigate if combination treatment with Bosentan and Sildenafil to patients with Eisenmenger syndrome is beneficial.
Surgical construction of an opening or window in the pericardium. It is often called subxiphoid pericardial window technique.
A developmental abnormality in which the spiral (aortopulmonary) septum failed to completely divide the TRUNCUS ARTERIOSUS into ASCENDING AORTA and PULMONARY ARTERY. This abnormal communication between the two major vessels usually lies above their respective valves (AORTIC VALVE; PULMONARY VALVE).
An autosomal recessive disorder characterized by RETINITIS PIGMENTOSA; POLYDACTYLY; OBESITY; MENTAL RETARDATION; hypogenitalism; renal dysplasia; and short stature. This syndrome has been distinguished as a separate entity from LAURENCE-MOON SYNDROME. (From J Med Genet 1997 Feb;34(2):92-8)
Rare, autosomal recessive disorder caused by deficiency of the beta 2 integrin receptors (RECEPTORS, LEUKOCYTE-ADHESION) comprising the CD11/CD18 family of glycoproteins. The syndrome is characterized by abnormal adhesion-dependent functions, especially defective tissue emigration of neutrophils, leading to recurrent infection.
Fenestra of the cochlea, an opening in the basal wall between the MIDDLE EAR and the INNER EAR, leading to the cochlea. It is closed by a secondary tympanic membrane.
Congenital conditions are those which are present from birth. They include structural deformities or loss of function in organs such as the <!--LGfEGNT2Lhm-->heart, gut or skeletal system. They can be corrected by <!--LGfEGNT2Lhm-->surgery, m...
Cardiovascular disease (CVD)
Acute Coronary Syndromes (ACS) Blood Cardiovascular Dialysis Hypertension Stent Stroke Vascular Cardiovascular disease (CVD) includes all the diseases of the heart and circulation including coronary heart disease (angina...
Pulmonary relating to or associated with the lungs eg Asthma, chronic bronchitis, emphysema, COPD, Cystic Fibrosis, Influenza, Lung Cancer, Pneumonia, Pulmonary Arterial Hypertension, Sleep Disorders etc Follow and track Lung Cancer News ...