Familial Primary Pulmonary Hypertension

Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.

Also Known As:

Heritable Pulmonary Arterial Hypertension; Idiopathic Pulmonary Arterial Hypertension; Idiopathic Pulmonary Hypertension; Pph1 With Hht; Primary Pulmonary Hypertension; Pulmonary Hypertension, Primary, 1; Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia; Pulmonary Hypertension, Primary, Dexfenfluramine-Associated; Pulmonary Hypertension, Primary, Fenfluramine-Associated; Hypertension, Idiopathic Pulmonary; Hypertension, Primary Pulmonary; Pulmonary Hypertension, Idiopathic; Pulmonary Hypertension, Primary

Networked: 1767 relevant articles (136 outcomes, 155 trials/studies)

Relationship Network

Disease Context: Research Results

Respiratory Tract Diseases: 6015
- Lung Diseases: 22246
  - Pulmonary Hypertension: 16678
    - Familial Primary Pulmonary Hypertension: 1767

Related Diseases

1.	Pulmonary Hypertension
2.	Pulmonary Arterial Hypertension
3.	Congenital Heart Defects (Congenital Heart Defect)
4.	Eisenmenger Complex (Eisenmenger's Syndrome)
5.	Connective Tissue Diseases (Connective Tissue Disease)

Experts

1.	Humbert, Marc: 34 articles (07/2021 - 02/2002)
2.	Morrell, Nicholas W: 27 articles (12/2021 - 04/2002)
3.	Matsubara, Hiromi: 19 articles (11/2020 - 03/2002)
4.	Seeger, Werner: 18 articles (01/2022 - 05/2002)
5.	Humbert, M: 18 articles (08/2012 - 06/2000)
6.	Wang, Lan: 17 articles (01/2022 - 08/2014)
7.	Simonneau, Gérald: 17 articles (01/2020 - 02/2002)
8.	Wilkins, Martin R: 16 articles (12/2021 - 03/2002)
9.	Yuan, Jason X-J: 16 articles (12/2021 - 09/2004)
10.	Simonneau, G: 16 articles (11/2009 - 02/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Familial Primary Pulmonary Hypertension:

1.	Epoprostenol (Prostacyclin)FDA LinkGeneric 07/01/2008 - "This service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected patients, particularly those with idiopathic pulmonary arterial hypertension for whom chronic intravenous prostacyclin remains the most effective medication. " 08/10/2004 - "Survival for children with idiopathic pulmonary arterial hypertension has significantly improved with CCB and epoprostenol. " 09/15/1994 - "Continuous intravenous prostacyclin resulted in sustained clinical and hemodynamic improvement and probably in improved survival in patients with severe primary pulmonary hypertension. " 04/01/1990 - "Prostacyclin produces substantial and sustained hemodynamic and symptomatic responses in severe primary pulmonary hypertension and may be useful in the management of some patients with this disease." 12/01/2016 - "Intravenous epoprostenol is an effective treatment for idiopathic and heritable pulmonary arterial hypertension. "
2.	Sildenafil Citrate (Viagra)FDA Link 09/01/2005 - "These data suggest that sildenafil may be useful in the management of idiopathic pulmonary arterial hypertension. " 04/07/2004 - "In a randomized, double-blind, crossover design, we compared the efficacy of sildenafil with placebo in patients with primary pulmonary hypertension (PPH). " 03/01/2010 - "Sildenafil is indicated in idiopathic pulmonary hypertension, although data have been extrapolated mainly from adult trial (class I, level of evidence A, extrapolated). " 07/01/2002 - "A study of clinical efficacy of sildenafil in patients with primary pulmonary hypertension." 11/01/2017 - "A 32-year-old African American woman with a history of primary pulmonary hypertension and 5-year history of oral sildenafil (Revatio) use presented with decreasing central vision in her left eye. "
3.	Vasodilator Agents (Vasodilators)IBA 01/01/2003 - "A 37-year-old woman with primary pulmonary hypertension and worsening symptomatology underwent pulmonary artery (PA) catheterization and vasodilator trials. " 05/01/1991 - "To determine correlates of acute vasodilator responsiveness in primary pulmonary hypertension, we retrospectively studied 25 patients, comparing 41 resting echo/Doppler and nine resting catheterization variables with the maximal reduction in pulmonary vascular resistance achieved during vasodilator trials. " 09/01/1988 - "The risk-benefit ratio in the use of vasodilators in the long-term treatment of primary pulmonary hypertension needs to be evaluated by a controlled trial, conducted in those who respond acutely. " 10/01/1984 - "Because of occasional beneficial responses, a trial of vasodilators is warranted in patients with primary pulmonary hypertension. " 08/05/2022 - "He was evaluated elsewhere and was being treated as primary pulmonary hypertension with pulmonary vasodilators. "
4.	Bosentan (Tracleer)FDA Link 03/01/2006 - "Data on long-term efficacy of bosentan in unselected idiopathic pulmonary arterial hypertension (IPAH) patients are lacking. " 01/01/2013 - "To study the effect of bosentan on the clinical status and cellular immunity of patients with idiopathic pulmonary hypertension (IPH). " 10/06/2001 - "In this double-blind, placebo-controlled study, 32 patients with pulmonary hypertension (primary or associated with scleroderma) were randomly assigned to bosentan (62.5mg taken twice daily for 4 weeks then 125 mg twice daily) or placebo for a minimum of 12 weeks. " 01/01/2019 - "Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art." 07/15/2013 - "Relationship between baseline ET-1 plasma levels and outcome in patients with idiopathic pulmonary hypertension treated with bosentan."
5.	Calcium Channel Blockers (Blockers, Calcium Channel)IBA 07/09/1992 - "This study suggests that high doses of calcium-channel blockers in patients with primary pulmonary hypertension who respond with reductions in pulmonary-artery pressure and pulmonary vascular resistance may improve survival over a five-year period." 01/01/2023 - "Long-term outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH) treated with calcium channel blockers (CCBs) are not well documented. " 01/01/2023 - "The Long-Term Response to Treatment with Calcium Channel Blockers in Patients with Idiopathic Pulmonary Arterial Hypertension." 01/01/2023 - "Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension." 09/01/2022 - "Calcium channel blockers (CCB), the first accepted treatment, is effective only in a small number of idiopathic pulmonary arterial hypertension (I-PAH) patients with vasoreactivity [these patients are identified by a positive acute pulmonary vasoreactive test (AVT) response]. "
6.	Endothelin Receptor AntagonistsIBA 12/01/1998 - "Endothelin antagonists also offer promise in a rapidly fatal condition, primary pulmonary hypertension. " 01/01/2010 - "[Using nonselective endothelin receptor antagonist in idiopathic pulmonary hypertension]." 07/01/2004 - "[Updated treatment of primary pulmonary hypertension -- the role of endothelin receptor antagonists]." 01/01/2008 - "The efficacy of endothelin receptor antagonists in Eisenmenger syndrome, which has similar pathophysiology to idiopathic pulmonary hypertension, remains unknown.We" 02/01/2009 - "Endothelin receptor antagonists (ERAs) have recently become prominent therapies for pulmonary arterial hypertension, and are being explored clinically in several areas, including resistant hypertension, idiopathic pulmonary fibrosis, and cancer. "
7.	Nitric Oxide (Nitrogen Monoxide)FDA Link 06/01/2015 - "Inhaled nitric oxide (INO) is cleared for the treatment of primary pulmonary hypertension in newborns. " 09/01/1994 - "Low dose (3 ppm) inhaled nitric oxide caused selective pulmonary vasodilation with improved systemic arterial pressure, cardiac index and arterial oxygenation in an infant with primary pulmonary hypertension. " 08/01/2000 - "The purpose of this study was to investigate the responses of patients with primary pulmonary hypertension (PPH) to constant work rate exercise and to examine the effect of nitric oxide (NO) inhalation. " 02/19/1994 - "Recently, nitric oxide (NO) has been recognized as an important endothelial factor with potent vasorelaxing properties; preliminary studies in patients with ARDS and in those with severe primary pulmonary hypertension have corroborated the power of this molecule in improving gas exchange and pulmonary hemodynamics without deleterious effects on the systemic circulation. " 12/01/2018 - "RV function improvement following nitric oxide inhalation demonstrated by gated blood pool SPECT in a patient with primary pulmonary hypertension."
8.	Nifedipine (Adalat)FDA LinkGeneric 11/01/1999 - "Echocardiographic predictors of an adverse response to a nifedipine trial in primary pulmonary hypertension: diminished left ventricular size and leftward ventricular septal bowing." 03/01/1985 - "Acute effect of sublingual nifedipine on right ventricular systolic time intervals in primary pulmonary hypertension--an echocardiographic study." 07/01/2018 - "Sustained response to low-dose nifedipine in a patient with idiopathic pulmonary arterial hypertension." 12/01/1996 - "Sustained improvement of pulmonary haemodynamics with low-dose of nifedipine in primary pulmonary hypertension." 05/01/1996 - "Although most demonstrate an initial response at readily attainable nifedipine concentrations with conventional dosages, a subset of patients seem to display delayed or incomplete oral absorption; these results may facilitate the clinical use of nifedipine in patients with primary pulmonary hypertension."
9.	Iloprost (Ventavis)FDA Link 01/01/2004 - "While inhaled iloprost has been described to be highly effective in the treatment of primary pulmonary hypertension, the possibility of contrary "paradoxical" reactions in isolated patients is emphasised, with a dramatic increase of PVR and a possible adverse outcome." 10/01/2010 - "[Efficacy of long-term oral monotherapy and additional effect of inhaled iloprost in patients with severe idiopathic pulmonary arterial hypertension]." 03/01/2015 - "In this study, 126 patients with PH were classified according to current guidelines (59, 34, 29, and 4 patients in groups 1/1', 3, 4, and 5, respectively; 20 patients had idiopathic pulmonary arterial hypertension [iPAH]), were randomly assigned to inhale iloprost 2.5 [Formula: see text]g (n = 67) or 5.0 [Formula: see text]g (n = 59) via the I-neb AAD system, and were assessed by right heart catheterization. " 08/01/2012 - "Iloprost has been used to test acute pulmonary vasoreactivity in idiopathic pulmonary arterial hypertension (PAH). " 06/01/2007 - "Impaired peripheral endothelial function in severe idiopathic pulmonary hypertension correlates with the pulmonary vascular response to inhaled iloprost."
10.	ProstaglandinsIBA 01/01/2007 - "[Effectiveness of prostaglandin treatment in idiopathic pulmonary hypertension]." 09/01/2003 - "In primary pulmonary hypertension, aerosolized prostanoids selectively reduce pulmonary vascular resistance and improve right ventricular function. " 06/01/2003 - "The pathogenesis of primary pulmonary hypertension in children remains as yet unclear, although treatment with prostanoids has proven effectively to improve the long-term prognosis." 11/01/2001 - "Increased levels of prostaglandin D(2) suggest macrophage activation in patients with primary pulmonary hypertension." 04/01/2013 - "Given the similarity of the pathology with idiopathic pulmonary arterial hypertension (IPAH) and ES, drugs that are usually reserved for IPAH such as prostanoids and phosphodiesterase type 5 inhibitors may be beneficial in the management of ES. "

Therapies and Procedures

1.	Therapeutics 09/01/2020 - "In PoPH, several medical therapies used in idiopathic pulmonary hypertension have been shown improve pulmonary hemodynamics, symptoms, and potentially LT outcomes; however, further study is needed to determine best treatment regimens, long-term outcomes on medical therapy, and role of LT. " 11/01/1989 - "This combination therapy may be effective in selected patients with primary pulmonary hypertension." 11/01/2014 - "Improved survival of Korean patients with idiopathic pulmonary arterial hypertension after the introduction of targeted therapies." 01/01/2013 - "Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH-specific therapies. " 04/01/1995 - "There was improved long-term survival in the 13 patients who survived blade balloon atrial septostomy compared with similar groups of primary pulmonary hypertension patients who received standard therapy (P < .05). "
2.	Intravenous Infusions 04/01/1996 - "Continuous intravenous infusion of PGI2 is an effective treatment of primary pulmonary hypertension in terms of exercise capacity and survival rate. " 04/10/2007 - "The goal of this study was to investigate the feasibility, safety, and initial clinical outcome of intravenous infusion of autologous endothelial progenitor cells (EPCs) in patients with idiopathic pulmonary arterial hypertension (IPAH). " 01/01/2001 - "Continuous intravenous infusion of prostacyclin is an effective treatment for primary pulmonary hypertension. " 06/22/2000 - "Continuous intravenous infusion of epoprostenol (prostacyclin) is an effective treatment for primary pulmonary hypertension. " 03/01/2000 - "Continuous intravenous infusion of prostacyclin is an effective treatment for primary pulmonary hypertension (PPH), and has recently been shown to be of benefit in PH associated with scleroderma (SSc). "
3.	Lung Transplantation 09/27/2016 - "Compared preoperative with postoperative, pulmonary artery systolic pressure from preoperative 128.2±25.6 mmHg dropped to postoperative 39.7±14.8 mmHg; pulmonary artery mean pressure from 75.5±13.94 mmHg to 19.61±6.43 mmHg; right atrial pressure from 21.5±2.43 mmHg to 5.22±4.18 mmHg, all P<0.000 1. Conclusion: Bilateral lung transplantation is an effective treatment for severe idiopathic pulmonary arterial hypertension, which has longer survival." 12/01/2013 - "An oversized allograft is associated with improved survival after lung transplantation for idiopathic pulmonary arterial hypertension." 06/01/2001 - "For recent decades, the medical treatment of primary pulmonary hypertension(PH) have shown an improved outcome as a bridge to lung transplantation. " 09/27/2016 - "Objective: To study the clinical efficacy of bilateral lung transplantation treating for severe idiopathic pulmonary hypertension (IPAH). " 09/01/2009 - "To study the effect of the LAS on lung transplantation for idiopathic pulmonary arterial hypertension (IPAH) compared with other major diagnoses. "
4.	Transplantation 04/10/2007 - "Transplantation of autologous endothelial progenitor cells may be beneficial in patients with idiopathic pulmonary arterial hypertension: a pilot randomized controlled trial." 04/22/2014 - "Response to letter regarding article, "transplantation for idiopathic pulmonary arterial hypertension: improvement in the lung allocation score era"." 04/22/2014 - "Letter by Eberlein and Reed regarding article, "transplantation for idiopathic pulmonary arterial hypertension: improvement in the lung allocation score era"." 06/25/2013 - "Transplantation for idiopathic pulmonary arterial hypertension: improvement in the lung allocation score era." 09/01/2008 - "Safety and efficacy of autologous endothelial progenitor cells transplantation in children with idiopathic pulmonary arterial hypertension: open-label pilot study."
5.	Heart-Lung Transplantation 10/01/2008 - "With our current pulmonary protection and immunosuppression, the long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension is excellent." 10/01/2008 - "The survival after lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension has been reportedly the lowest among the major diagnostic categories of lung transplant recipients. " 10/01/2008 - "Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension." 04/01/1999 - "Thirty-nine patients had heart-lung transplantation for primary pulmonary hypertension. " 04/01/1999 - "We reviewed our institution's experience with heart-lung transplantation for primary pulmonary hypertension. "