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We present a 10 year old boy with syncope who was found to have long-QT syndrome and severe pulmonary hypertension (PH) both in the absence of a secondary cause, to our knowledge this is the first report with this unusual coexistence. His genetic tests were positive for hereditary hemorrhagic telangiectasia and long QT syndrome (LQTS) without any family history of PH or LQTS. We demonstrated that digital subtraction pulmonary angiography was more useful compared to CT angiogram to demonstrate pulmonary vascular changes which correlated with a no-response to acute vasoreactivity testing during right heart catheterization. He has been stable for the last 2 years on ambrisentan, sildenafil and nadolol without recurrence of symptoms.
This article was published in the following journal.
Name: Cardiovascular & hematological agents in medicinal chemistry
The Pulmonary Embolism in Syncope Italian Trial reported 17.3% prevalence of pulmonary embolism (PE) in patients admitted with syncope. We investigated the prevalence of venous thromboembolism [VTE, i...
Antiphospholipid syndrome (APS) is an acquired thrombophilia with an uncertain role in the development of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to assess the association of A...
Approximately one in six patients hospitalized with syncope have pulmonary embolism (PE), according to the PE in Syncope Italian Trial study. Subsequent studies using administrative data have reported...
BACKGROUND Pulmonary hypertension is a common complication of interstitial lung disease. This study was conducted to retrospectively analyze the incidence of pulmonary hypertension among interstitial ...
Chronic hypoxia causes sustained pulmonary vasoconstriction and vascular remodeling leading to development of pulmonary hypertension in high-altitude residents. Although pulmonary hypertension is of m...
Syncope is a rapid onset, transient, loss of consciousness with a short duration. This symptom has been reported to be a specific presentation of patients with pulmonary embolism. However,...
As a pulmonary hypertension center, we have created a database that prospectively tracks patients with different forms of secondary pulmonary hypertension. Records include genetic analysi...
Physical activity is an important factor in understanding how diseases can affect a child. Decreases in physical activity are sometimes the first thing that happens before a child is diagn...
OBJECTIVES: To evaluate syncope recurrence at follow up in patients with a positive cardio inhibitory response to the head up tilt testing (HUT) evaluation. METHODS: 36 consecutive patien...
The primary objective is to establish the safety of autologous progenitor cell-based gene therapy of heNOS in patients with severe Pulmonary Arterial Hypertension(PAH) refractory to conven...
Hypertrophy and dilation of the RIGHT VENTRICLE of the heart that is caused by PULMONARY HYPERTENSION. This condition is often associated with pulmonary parenchymal or vascular diseases, such as CHRONIC OBSTRUCTIVE PULMONARY DISEASE and PULMONARY EMBOLISM.
Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.
A prostaglandin that is a powerful vasodilator and inhibits platelet aggregation. It is biosynthesized enzymatically from PROSTAGLANDIN ENDOPEROXIDES in human vascular tissue. The sodium salt has been also used to treat primary pulmonary hypertension (HYPERTENSION, PULMONARY).
A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)
A malformation of the heart in which the embryonic common PULMONARY VEIN was not incorporated into the LEFT ATRIUM leaving behind a perforated fibromuscular membrane bisecting the left atrium, a three-atrium heart. The opening between the two left atrium sections determines the degree of obstruction to pulmonary venous return, pulmonary venous and pulmonary arterial hypertension.
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...
Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder characterized by abnormally high blood pressure in the arteries between the heart and lungs of affected individuals. Symptoms can range from mild breathles...
Bioinformatics is the application of computer software and hardware to the management of biological data to create useful information. Computers are used to gather, store, analyze and integrate biological and genetic information which can then be applied...