Infundibular Pulmonary Stenosis Treatment & Management

Updated: Nov 23, 2022
  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
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Treatment

Medical Care

The primary function of medical management is to assess the severity and progression of stenosis. Note the following:

  • A decision must be made as to when the gradient is sufficiently severe to warrant relieving the obstruction.

  • Careful follow-up is necessary for children younger than 12 years because changes in the severity of their conditions are not uncommon.

  • Follow-up is conducted almost wholly by Doppler studies.

  • If diagnostic questions arise after careful Doppler echocardiographic evaluation, cardiac catheterization and angiography are sometimes performed before surgical intervention. However, transesophageal echocardiogram and magnetic resonance imaging have largely replaced the role of diagnostic catheterization in these patients. [8]

  • The main indicators of progression are the degree of right ventricular hypertrophy on ECG and echocardiography and the peak and mean systolic pressure gradients across the pulmonary outflow. Onset of symptoms (eg, hypoxic spells, angina, signs of congestive heart failure, radiological evidence of cardiomegaly) also influences the decision to surgically intervene.

  • Heart failure therapy is indicated only as a temporary measure in patients with heart failure. Supervise heart failure therapy closely to look for signs of worsening. Ensure adequate hydration and, if necessary, use beta-adrenergic blockers to relax the infundibular muscles. [9]

  • Balloon dilatation and stenting of the right ventricular outflow tract can be considered in babies with significant infundibular pulmonary stenosis (IPS). [10]

  • Prior to intervention, administration of alprostadil minimizes cyanosis in neonates with ductal-dependent pulmonary blood flow.

Transfer

When age appropriate, transfer the patient to a cardiologist specializing in adults.

Subsequent hospitalization is required only for cardiac catheter studies, when indicated, or for surgical intervention.

Diet and activity

The severity of malnutrition determines dietary requirements.

No activity restrictions are necessary for patients with mild-to-moderate stenosis. Allow patients to limit their own activity, based on personal tolerance.

Avoid dehydration at all costs.

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Surgical Care

In general, surgical interventions for patients with infundibular pulmonary stenosis (IPS) include the following:

  • Corrective surgery, specifically resection of the hypertrophied infundibulum and enlargement of outflow tract with patch, if required

  • Conduit repair of outflow for severe cases in infancy (Some have achieved good results using a conduit to bypass the obstructed outflow segment.)

  • Insertion of a Blalock-Taussig shunt (used only for infants with an inoperable lesion or in cases in which surgical skills are not available)

Indications for surgical intervention include the following:

  • Presence of such symptoms as shortness of breath, cyanosis, chest pain

  • Severe stenosis with gradients exceeding 100 mm Hg, even if the patient is asymptomatic

  • Severe cyanosis or heart failure in infancy

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Consultations

Consider consultations with the following specialists:

  • Pediatric cardiologist

  • Radiologist

  • Family physician

  • Occupational therapist

  • Physiotherapist

  • Psychologist

  • Pharmacist

  • Dietitian

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