Skip to content

Pulmonary Hypertension: Recognition & Referral

Learning Objectives

By the end of this rotation, you should be able to:

  1. Recognize symptoms and signs suggesting pulmonary hypertension
  2. Identify at-risk populations who need screening
  3. Know when to refer to pediatric cardiology/pulmonary hypertension specialist
  4. Understand basic management principles for hospitalized patients with known PH

What Is Pulmonary Hypertension?

Definition: Mean pulmonary artery pressure >20 mmHg at rest (by catheterization)

Key concept: Elevated pressure in the pulmonary circulation that can lead to right heart failure.

Symptoms and Signs

Symptoms (Often Nonspecific)

Symptom Notes
Exertional dyspnea Most common; progressive
Exercise intolerance May be attributed to "out of shape"
Syncope/near-syncope with exertion Concerning - indicates severe PH
Chest pain with exertion RV ischemia
Fatigue Nonspecific

Physical Exam Findings

Finding What It Indicates
Loud P2 (palpable) Elevated PA pressure
RV heave RV hypertrophy
TR murmur RV dilation
Hepatomegaly Right heart failure
Peripheral edema Right heart failure
Cyanosis Shunting (if present)

At-Risk Populations - Screen These Patients

Population Why They're at Risk
CHD with shunts (especially if delayed repair) Eisenmenger risk
BPD/chronic lung disease Group 3 PH
Sickle cell disease Multiple mechanisms
Connective tissue disease Scleroderma, lupus, MCTD
Congenital diaphragmatic hernia Pulmonary hypoplasia
Down syndrome Higher risk with CHD
Family history of PAH Heritable PAH

When to Refer to Cardiology

Urgent Referral

  • Syncope or near-syncope with exertion
  • Signs of right heart failure
  • Known PH with worsening symptoms
  • Unexplained cyanosis

Non-Urgent Referral

  • Unexplained exertional dyspnea after pulmonary workup
  • At-risk patient with suggestive symptoms
  • Echo suggesting elevated PA pressures

Managing Hospitalized Patients with Known PH

General Principles

Do Don't
Call PH specialist early Don't start or stop PH meds without them
Maintain oxygen saturation Don't give excessive fluids
Treat underlying illness Don't use vasoconstrictors if possible
Prevent hypoxia/acidosis Don't delay treatment of fever/infection

Medications You'll See

Patients with PH may be on specialized medications: - Sildenafil/tadalafil - PDE5 inhibitors - Bosentan/ambrisentan - Endothelin receptor antagonists - Epoprostenol (Flolan) - IV prostacyclin (continuous infusion) - Treprostinil - Subcutaneous or IV prostacyclin

Critical: Never stop prostacyclin infusions - can cause rebound PH crisis.

PH Crisis - Recognize and Get Help

Signs of PH crisis: - Severe hypoxia - Hypotension - RV failure (elevated CVP, hepatomegaly) - Syncope

Immediate actions: 1. Call for help (cardiology, PICU) 2. Oxygen - maintain saturations 3. Avoid agitation - sedation may be needed 4. Inhaled nitric oxide if available 5. Treat underlying trigger (hypoxia, acidosis, pain)

Key Teaching Points for Residents

  1. Exertional syncope is always concerning - rule out PH and other cardiac causes
  2. Loud P2 on exam should prompt cardiology referral
  3. Screen at-risk populations - especially CHD, BPD, sickle cell
  4. Never stop prostacyclin infusions - can be fatal
  5. PH patients are fragile - call specialists early when ill
  6. Avoid hypoxia, acidosis, and agitation in known PH patients

Key Guidelines

2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension Eur Heart J. 2022;43(38):3618-3731 PMID: 36017548

2019 AHA/ATS Pediatric Pulmonary Hypertension Guidelines Circulation. 2019;140(24):e875-e877 PMID: 31814400

Board Pearls

Pearl: Exertional syncope + loud P2 = think pulmonary hypertension

This combination suggests severe PH. Refer urgently - these patients can decompensate quickly.

Pearl: NEVER stop prostacyclin infusions in PH patients

Abrupt discontinuation can cause fatal rebound PH crisis. If pump malfunctions, get help immediately.

Pearl: CHD with shunts → screen for Eisenmenger physiology

Late repair of VSD/PDA/ASD can lead to irreversible pulmonary vascular disease. Look for cyanosis and clubbing.

Self-Assessment

Question 1

Which physical exam finding is most suggestive of pulmonary hypertension?

Answer **Loud, palpable P2** A loud second heart sound (P2 component) indicates elevated pulmonary artery pressure. When P2 is palpable at the left upper sternal border, this strongly suggests significant PH.

Question 2

A child with known pulmonary hypertension on continuous IV epoprostenol (Flolan) is admitted for pneumonia. The IV pump malfunctions. What is the immediate priority?

Answer **NEVER stop the prostacyclin - this is an emergency** Abrupt discontinuation of prostacyclin can cause fatal rebound PH crisis. Call for immediate help and work to restore the infusion. If the central line is the problem, the drug can temporarily be given peripherally while access is obtained.

Question 3

Which patient population should be routinely screened for pulmonary hypertension?

Answer **Patients at high risk for PH development:** - CHD with shunts (especially if repair delayed) - Bronchopulmonary dysplasia (BPD) - Sickle cell disease - Connective tissue diseases (scleroderma, lupus) - Congenital diaphragmatic hernia - Down syndrome with CHD - Family history of heritable PAH