What are the presentations of a complication of pulmonary hypertension?

Complications of Pulmonary Hypertension: Presentations

The most common presentations of pulmonary hypertension complications include right ventricular failure with peripheral edema, ascites, and elevated jugular venous pressure, as well as mechanical complications such as hemoptysis, hoarseness, and pulmonary artery dissection or rupture. 1

Right Ventricular Dysfunction Manifestations

• Progressive right ventricular failure is the primary complication of pulmonary hypertension, presenting with:

  • Elevated jugular venous pressure with prominent V waves 1
  • Hepatomegaly and pulsatile liver 1
  • Peripheral edema (present in 32% of patients) 1
  • Ascites 1
  • Cool extremities due to low cardiac output and peripheral vasoconstriction 1
  • Abdominal distension from hepatic congestion 1
  • Fatigue and weakness due to decreased cardiac output 1

• Advanced right ventricular failure signs include:

  • Right ventricular S3 gallop (present in 23% of patients) 1
  • Hypotension and diminished pulse pressure 1
  • Syncope, especially with exertion 1

Mechanical Complications

• Pulmonary arterial dilatation can lead to several mechanical complications:
  • Hemoptysis due to rupture of hypertrophied bronchial arteries 1
  • Hoarseness from compression of the left recurrent laryngeal nerve 1
  • Wheeze caused by large airway compression 1
  • Angina due to myocardial ischemia from compression of the left main coronary artery 1, 2
  • Pulmonary artery rupture or dissection leading to cardiac tamponade, a potentially fatal complication 1

Arrhythmias

• Supraventricular arrhythmias occur in advanced disease:
  • Atrial flutter is most common 1
  • Atrial fibrillation occurs with a cumulative incidence of 25% after 5 years 1
  • These arrhythmias compromise cardiac output and almost invariably lead to clinical deterioration 1
  • Ventricular arrhythmias are rare but may occur 1

Pleural and Pericardial Complications

• Pleuritic chest pain may result from:
  • Pleural inflammation secondary to pulmonary congestion 2
  • Pericardial inflammation due to right ventricular strain 2
  • Pleural effusions, especially in advanced disease 2, 3

Pulmonary Veno-Occlusive Disease Complications

• PVOD is a rare but serious complication of pulmonary hypertension presenting with:
  • Pleuritic chest pain due to pleural inflammation 2
  • Severe hypoxemia and lower diffusion capacity compared to other forms of pulmonary hypertension 3
  • Digital clubbing (uncommon in other forms of pulmonary hypertension) 3
  • Pulmonary edema, especially when treated with pulmonary vasodilators 2, 3

Thromboembolic Complications

• Pulmonary embolism can present as a complication or cause of pulmonary hypertension:
  • Pleuritic chest pain due to pleural irritation 2
  • Worsening hypoxemia 2
  • Progressive dyspnea 4
  • Can lead to chronic thromboembolic pulmonary hypertension (CTEPH) 2, 4

Treatment-Related Complications

• Complications from epoprostenol (prostacyclin) therapy:
  • Flushing, headache, hypotension, tachycardia 5
  • Nausea, vomiting, and diarrhea 5
  • In overdose cases: hypoxemia, hypotension, and respiratory arrest (potentially fatal) 5

Clinical Pearls and Pitfalls

• Right ventricular failure is the main cause of death in pulmonary hypertension 6
• The presence of digital clubbing should raise suspicion for PVOD, congenital heart disease, interstitial lung disease, or liver disease rather than idiopathic pulmonary arterial hypertension 1
• Intubation should be avoided if possible in patients with right ventricular failure, as both hypoxemia and hypercapnia can worsen right ventricular function 7
• In patients with right ventricular failure and shock, vasopressors and inotropes are often required rather than fluid boluses, which can exacerbate right ventricular ischemia 7
• Early consultation with pulmonary hypertension specialists is advised for patients with complications, as they may require specialized interventions not readily available in all settings 7