sildenafil-citrate and Telangiectasia--Hereditary-Hemorrhagic

sildenafil-citrate has been researched along with Telangiectasia--Hereditary-Hemorrhagic* in 2 studies

Other Studies

2 other study(ies) available for sildenafil-citrate and Telangiectasia--Hereditary-Hemorrhagic

Article	Year
Syncope in a Child with Pulmonary Hypertension and Positive Gene Tests for Hereditary Hemorrhagic Telangiectasia and Long QT Syndrome. Cardiovascular & hematological agents in medicinal chemistry, 2020, Volume: 18, Issue:1 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 noresponse 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. Topics: Angiography; Child; Echocardiography; Electrocardiography; Genetic Testing; Humans; Hypertension, Pulmonary; Long QT Syndrome; Male; Nadolol; Phenylpropionates; Pyridazines; Sildenafil Citrate; Syncope; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed	2020
Pulmonary hypertension in a patient with hereditary haemorrhagic telangiectasia. BMJ case reports, 2013, Feb-01, Volume: 2013 A young male patient reported for evaluation of progressive easy fatigability, accompanied by a recent history of recurrent haemoptysis. His clinical examination was unremarkable except for evidence of pulmonary arterial hypertension (PAH). Routine investigations (haemogram, coagulogram, serological tests for connective tissue disorders and a sputum Ziehl Neelsen stain for acid-fast bacilli) were normal. Two-dimensional echocardiography suggested PAH (pulmonary artery systolic pressure-67 mm Hg), whereas the 64-slice spiral CT pulmonary angiogram showed a dilated main pulmonary artery along with bilateral arteriovenous malformations. Cardiac catheterisation performed subsequently confirmed the presence of PAH. On the basis of the above findings, a diagnosis of hereditary haemorrhagic telangiectasia (HHT) complicated with PAH was made, and the patient was started on oral sildenafil therapy to which he responded well. This rare complication of HHT, which requires a high degree of suspicion for diagnosis, is discussed. Topics: Echocardiography; Familial Primary Pulmonary Hypertension; Fatigue; Hemoptysis; Humans; Hypertension, Pulmonary; Male; Piperazines; Purines; Sildenafil Citrate; Sulfones; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed; Vasodilator Agents; Young Adult	2013

Article

Year

Syncope in a Child with Pulmonary Hypertension and Positive Gene Tests for Hereditary Hemorrhagic Telangiectasia and Long QT Syndrome.

Cardiovascular & hematological agents in medicinal chemistry, 2020, Volume: 18, Issue:1

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 noresponse 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.

Topics: Angiography; Child; Echocardiography; Electrocardiography; Genetic Testing; Humans; Hypertension, Pulmonary; Long QT Syndrome; Male; Nadolol; Phenylpropionates; Pyridazines; Sildenafil Citrate; Syncope; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed

2020

Pulmonary hypertension in a patient with hereditary haemorrhagic telangiectasia.

BMJ case reports, 2013, Feb-01, Volume: 2013

A young male patient reported for evaluation of progressive easy fatigability, accompanied by a recent history of recurrent haemoptysis. His clinical examination was unremarkable except for evidence of pulmonary arterial hypertension (PAH). Routine investigations (haemogram, coagulogram, serological tests for connective tissue disorders and a sputum Ziehl Neelsen stain for acid-fast bacilli) were normal. Two-dimensional echocardiography suggested PAH (pulmonary artery systolic pressure-67 mm Hg), whereas the 64-slice spiral CT pulmonary angiogram showed a dilated main pulmonary artery along with bilateral arteriovenous malformations. Cardiac catheterisation performed subsequently confirmed the presence of PAH. On the basis of the above findings, a diagnosis of hereditary haemorrhagic telangiectasia (HHT) complicated with PAH was made, and the patient was started on oral sildenafil therapy to which he responded well. This rare complication of HHT, which requires a high degree of suspicion for diagnosis, is discussed.

Topics: Echocardiography; Familial Primary Pulmonary Hypertension; Fatigue; Hemoptysis; Humans; Hypertension, Pulmonary; Male; Piperazines; Purines; Sildenafil Citrate; Sulfones; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed; Vasodilator Agents; Young Adult

2013