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Infundibular Pulmonary Stenosis Clinical Presentation

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
Updated: Feb 21, 2014


Infundibular pulmonary stenosis (IPS) manifestations depend on the severity of obstruction and presence or absence of associated cardiac anomalies. They may include the following:

  • Most children grow well and are asymptomatic, even when stenosis is moderate or severe.
  • The murmur is discovered on routine auscultation, usually at birth, although cyanosis may lead to discovery of maximum obstruction.
  • Symptoms are rare in infants, with the notable exception of patients with critical stenosis.
  • Subjective complaints tend to increase with age.
  • Dyspnea and fatigue are the most common symptoms.
  • Exertion may provoke syncope or even death.
  • Precordial pain is common, and epigastric pain is often present.
  • Frank right-sided heart failure occasionally occurs in infancy or early childhood.
  • Squatting is extremely rare in children with isolated PS (compared with tetralogy of Fallot)


Physical examination findings may include the following:

  • Growth and development are usually normal. Frank heart failure is rarely evident.
  • Chest asymmetry occasionally accompanies severe stenosis, but precordial bulge is uncommon.
  • Jugular venous pulse shows larger a waves as the degree of obstruction increases. These presystolic pulsations may be felt during palpation of the liver, even without evidence of cardiac failure.
  • Prominent left parasternal heave occurs if PS is significant.
  • A systolic thrill is present at the second and third left intercostal space near the sternum. Occasionally, the thrill may disappear with onset of failure.


The first heart sound is normal.

The pulmonic valve component (P-2) of the second heart sound is soft and delayed in moderate-to-severe stenosis. Note the following features:

  • The degree of split is proportionate to the severity of the obstruction; the greater the obstruction, the longer the RV takes to empty and the wider the split.
  • P-2 decreases in intensity in proportion to the pressure in the PA. The lower the pressure, the softer the P-2. P-2 may be inaudible with maximal obstruction.
  • In severe stenosis with unchanging cardiac output, the split may be fixed. A loud pansystolic crescendo-decrescendo murmur (ejection type), with its maximal intensity at mid systole or later (indistinguishable from that of isolated pulmonary valve stenosis [PVS]), is heard at the left sternal border and is well-conducted to the precordium, neck, and back.

A third heart sound is audible in the presence of an associated atrial septal defect (ASD) or anomalous pulmonary vein.

A fourth heart sound is heard at the lower left sternal border in severe cases. This fourth heart sound is associated with a large a wave in the RA and usually indicates a severe lesion.

Note the absence of the ejection click that characterizes valvar PS.

A loud, long, systolic crescendo-decrescendo murmur (ejection type), with its maximal intensity at mid systole or later (indistinguishable from that of isolated PVS), is heard at the left sternal border and is well-conducted to the precordium, neck, and back. Note the following features:

  • The murmur, although louder at the second and third left intercostal space, may be heard well at the low left sternal border.
  • The later the peak intensity of the murmur occurs, the greater the obstruction.
  • Although murmur loudness does not necessarily increase with severity, murmurs of less than grade 3/6 usually occur with mild stenosis. With moderate-to-severe stenosis, murmurs are usually systolic and grade 4/6 or louder.
  • The length of the murmur depends on duration of RV systole that, in turn, depends on severity of the stenosis. Thus, mild stenosis is associated with a short murmur, with its peak earlier than mid systole. In moderate stenosis, the murmur ends at or slightly after the aortic component of the second heart sound, which remains audible. With marked-to-severe obstruction, the murmur extends beyond the aortic component, which may be obscured.

Critical stenosis

Infants with critical stenosis present with variable findings, including the following:

  • Heart failure is prominent.
  • A small infant with maximal obstruction may have minimal murmur (sometimes overlooked) and cyanosis.
  • An additional systolic murmur is heard in the lower left parasternal region from the tricuspid regurgitation (TR).
  • Absence of P-2 along with the presence of cardiomegaly and the holosystolic murmur of TR highly suggests a critical PS diagnosis.


See Pathophysiology.

Contributor Information and Disclosures

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey Allen Towbin, MD, MSc FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

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Chest radiograph of a 2-year-old boy with severe pulmonary stenosis (infundibular). Note the mild cardiomegaly, reduced pulmonary vascularity, and absence of poststenotic dilatation of pulmonary artery.
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