Condition Lookup
Sub-Category:
Pulmonary Hypertension (PH)
Number of Conditions: 2
Idiopathic Pulmonary Arterial Hypertension (IPAH)
Specialty: Pulmonology
Category: Pulmonary Vascular Diseases
Sub-category: Pulmonary Hypertension (PH)
Symptoms:
shortness of breath; fatigue; chest pain; dizziness; fainting; swelling in ankles or legs (edema); palpitations; blue discoloration of lips or skin (cyanosis)
Root Cause:
Narrowing and stiffening of pulmonary arteries with no known cause, leading to increased pulmonary vascular resistance and high blood pressure in the lungs.
How it's Diagnosed: videos
Diagnosis is made using echocardiography, right heart catheterization (gold standard), pulmonary function tests, chest X-ray, CT scan, and blood tests to rule out other causes.
Treatment:
Treatment includes medications to lower pulmonary blood pressure, improve exercise capacity, and prevent disease progression; oxygen therapy; and in severe cases, lung transplantation.
Medications:
Medications include phosphodiesterase-5 inhibitors (e.g., sildenafil , tadalafil ), endothelin receptor antagonists (e.g., bosentan , ambrisentan , macitentan ), prostacyclin analogs (e.g., epoprostenol , treprostinil , iloprost ), soluble guanylate cyclase stimulators (e.g., riociguat ), and calcium channel blockers (e.g., nifedipine , diltiazem , only for specific cases).
Prevalence:
How common the health condition is within a specific population.
Rare; estimated prevalence is 1-2 cases per million annually, with a higher incidence in women aged 30-50 years.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Female gender, genetic predisposition (BMPR2 gene mutation), and autoimmune diseases.
Prognosis:
The expected outcome or course of the condition over time.
Variable; prognosis depends on the severity at diagnosis and response to therapy. Without treatment, survival is 2-3 years, but modern therapies improve long-term outcomes.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Right-sided heart failure, arrhythmias, blood clots, and potential death if untreated.
Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Specialty: Pulmonology
Category: Pulmonary Vascular Diseases
Sub-category: Pulmonary Hypertension (PH)
Symptoms:
shortness of breath; fatigue; chest pain; dizziness; fainting; swelling in ankles or legs (edema); persistent cough; reduced exercise tolerance
Root Cause:
Chronic obstruction and remodeling of pulmonary arteries due to unresolved blood clots, causing high blood pressure in the lungs.
How it's Diagnosed: videos
Diagnosed using echocardiography, right heart catheterization (gold standard), ventilation-perfusion (V/Q) scan, CT pulmonary angiography, and pulmonary angiography. Blood tests and other imaging may rule out other causes.
Treatment:
Treatment options include pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), anticoagulation therapy, oxygen therapy, and medications to reduce pulmonary vascular resistance.
Medications:
Medications include anticoagulants (e.g., warfarin , rivaroxaban , apixaban ), soluble guanylate cyclase stimulators (e.g., riociguat ), and prostacyclin analogs or endothelin receptor antagonists in inoperable cases.
Prevalence:
How common the health condition is within a specific population.
Rare; affects approximately 3-5% of individuals who have had a pulmonary embolism.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
History of acute pulmonary embolism, inadequate anticoagulation treatment, thrombophilia, splenectomy, or chronic inflammatory diseases.
Prognosis:
The expected outcome or course of the condition over time.
With successful pulmonary endarterectomy, prognosis is excellent. In inoperable cases, long-term medical management improves quality of life but prognosis varies.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Right-sided heart failure, arrhythmias, blood clots, and recurrent pulmonary embolism.