What are the symptoms and treatment options for pulmonary hypertension, particularly in patients with a history of heart disease, lung disease, or connective tissue disorders?

Symptoms of Pulmonary Hypertension

Pulmonary hypertension presents with non-specific symptoms primarily related to progressive right ventricular dysfunction, with exertional dyspnea being the most common presenting complaint. 1

Primary Symptoms

Exertional Symptoms (Most Common)

• Shortness of breath on exertion is the hallmark symptom and typically the initial manifestation 1, 2
• Fatigue and weakness during physical activity are common early complaints 1
• Exertional syncope should raise strong suspicion for pulmonary hypertension, particularly in younger patients 1, 2
• Exercise-induced nausea and vomiting occur less commonly but are characteristic 1

Cardiac-Related Symptoms

• Angina pectoris may occur due to right ventricular ischemia or compression of the left main coronary artery by dilated pulmonary arteries 1
• Palpitations reflect cardiac strain 3
• Dry cough is a frequent but non-specific complaint 1, 3

Advanced Disease Symptoms (Rest Symptoms)

• Dyspnea at rest indicates advanced disease and significant right ventricular failure 1
• Abdominal distension and ankle edema develop with progressive right ventricular failure 1
• Cool extremities reflect reduced cardiac output in severe cases 1

Physical Examination Findings

Cardiac Signs

• Left parasternal lift indicates right ventricular hypertrophy 1
• Accentuated pulmonary component of the second heart sound (loud P2) is a key finding 1, 4
• Right ventricular third heart sound (S3 gallop) suggests volume overload 1
• Pansystolic murmur of tricuspid regurgitation reflects elevated right ventricular pressures 1
• Diastolic murmur of pulmonary regurgitation indicates severe pulmonary hypertension 1

Signs of Right Heart Failure

• Elevated jugular venous pressure is a critical finding in advanced disease 1, 4
• Hepatomegaly develops with venous congestion 1
• Ascites indicates severe right-sided failure 1
• Peripheral edema (ankle swelling) is common but non-specific 1, 4

Respiratory Examination

• Absence of wheeze and crackles helps distinguish pulmonary hypertension from primary lung disease 1
• Presence of inspiratory crackles suggests underlying interstitial lung disease as the cause 1

Disease-Specific Presentations

Connective Tissue Disease-Associated Features

• Telangiectasia, digital ulceration, and sclerodactyly suggest systemic sclerosis as the underlying cause 1
• These findings are particularly important in patients with connective tissue disorders 5

Liver Disease-Associated Features

• Spider naevi, testicular atrophy, and palmar erythema suggest portopulmonary hypertension 1

Mechanical Complications (Rare but Important)

• Hemoptysis from rupture of hypertrophied bronchial arteries 1
• Hoarseness from compression of the left recurrent laryngeal nerve by dilated pulmonary artery 1
• Wheeze from large airway compression by dilated vessels 1
• Signs of cardiac tamponade if pulmonary artery rupture or dissection occurs 1

Clinical Context by Patient Population

Heart Disease Patients

• Up to 60% of patients with severe left ventricular systolic dysfunction develop pulmonary hypertension 1
• Up to 70% of patients with heart failure with preserved ejection fraction may present with pulmonary hypertension 1
• Virtually all patients with severe symptomatic mitral valve disease develop pulmonary hypertension 1

Lung Disease Patients

• Symptoms disproportionate to pulmonary function test results should prompt evaluation for pulmonary hypertension 1, 6
• Disproportionally low DLCO and low pCO2 are common indicators of pulmonary hypertension in lung disease patients 1
• Peripheral edema in lung disease patients may result from hypoxemia effects on the renin-angiotensin-aldosterone system, not just right ventricular failure 1

Critical Red Flags Requiring Urgent Evaluation

• Rapidly progressing symptoms warrant urgent specialist referral 3
• Syncope is a particularly ominous symptom requiring immediate evaluation 2, 3
• Signs of right heart failure (elevated JVP, hepatomegaly, ascites) indicate advanced disease 1, 6
• Symptoms at rest represent decompensated disease 1

Important Clinical Pitfalls

• Symptoms are non-specific and can be mistaken for deconditioning, anxiety, or primary cardiac/pulmonary disease 1, 7
• Physical examination has poor sensitivity for detecting moderate pulmonary hypertension, particularly in obese patients 4
• Lack of response to conventional treatment for dyspnea should prompt consideration of pulmonary hypertension as an alternative diagnosis 3
• Clinical presentation may be modified by underlying diseases causing pulmonary hypertension and other concurrent conditions 1