Right ventricular outflow tract obstruction natural history

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Right ventricular outflow tract obstruction Microchapters

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Overview

Anatomy of Pulmonary Valve

Classification

Pulmonary valve stenosis
Pulmonary subvalvular stenosis
Pulmonary supravalvular stenosis
Pulmonary atresia

Pathophysiology

Causes

Differentiating Right ventricular outflow tract obstruction from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

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Cardiac Catheterization

Pulmonary Angiography

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Surgery

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Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Pulmonary artery conduits/Prosthetic Valves

Double-Chambered Right Ventricle

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

Natural History

Patients with congenital pulmonary stenosis manifest clinical features few hours after birth, in childhood or in adulthood. Manifestation of symptoms, symptom severity and the outcomes are dependent on the severity of stenosis.[1] Patients with mild pulmonic stenosis have a benign course and do not progress and patients with moderate and severe stenosis have dyspnea with exertion and syncope.[2]

Complications

If left untreated, patients with moderate to severe stenosis progress to develop tricuspid regurgitation and right ventricular dysfunction leading to right ventricular failure and arrhythmias.[3]

Prognosis

Patients with moderate to severe pulmonic valve stenosis are managed well with surgery or balloon valvuloplasty and have very good prognosis. [4]

References

  1. Hayes CJ, Gersony WM, Driscoll DJ, Keane JF, Kidd L, O'Fallon WM; et al. (1993). "Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis". Circulation. 87 (2 Suppl): I28–37. PMID 8425320.
  2. Mody MR (1975). "The natural history of uncomplicated valvular pulmonic stenosis". Am Heart J. 90 (3): 317–21. PMID 1163423.
  3. Wolfe RR, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, Kidd L; et al. (1993). "Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring". Circulation. 87 (2 Suppl): I89–101. PMID 8425327.
  4. Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD; et al. (2008). "2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Circulation. 118 (15): e523–661. doi:10.1161/CIRCULATIONAHA.108.190748. PMID 18820172.

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