TOF and Absent Pulmonary Valve with Aneurysmal Right Ventricular
Outflow Tract
In the present issue of the journal, Mishra and collogues have presented
interesting images of an adult patient with Tetralogy of Fallot with
absent pulmonary valve and aneurysmally dilated right ventricular
outflow tract (1).
Survival of a patient diagnosed with Tetralogy of Fallot and absent
pulmonary valve into adulthood, is a rarity. Patients diagnosed with
this condition usually present in infancy with symptoms of respiratory
distress and tachypnea, mainly because of the tracheobronchial
compression resulting from massive dilatation of the main pulmonary
artery and its first and second order branches. The consequential
tracheomalacia and bronchomalacia determines the timing and severity of
respiratory compromise.
This patient presented at the age of 37 years with symptoms of dyspnea
without any history of cyanosis. Such a late presentation might have
been due to the insignificant airway compression by the dilated main and
branch pulmonary arteries. In such patients, the right ventricular
infundibulum is aneurysmally dilated because of long standing pulmonary
regurgitation and right ventricular outflow tract obstruction. Contrary
to what its name suggests, there is presence of rudimentary and
dysplastic pulmonary leaflets in this condition.
A one-stage repair is the preferred method of choice. Asymptomatic
patients are usually operated on at 6-12 months of age and symptomatic
patients are operated on a semi-urgent basis. Various surgical
approaches have been described to correct this anomaly. The closure of
ventricular septal defect, relief of right ventricular outflow tract
obstruction, and achievement of a competent pulmonary valve are the
primary goals of the surgery. These are followed by a specific aim to
decompress the airways from dilated pulmonary arteries, if required. In
adults, establishing competence of the pulmonary valve can be
comparatively easy as compared to an infant. Reconstruction of the right
ventricular outflow can be done using a bioprosthetic valve or hand sewn
valve leaflets.
1. Mishra AK, Rana P, Raja J, Bansal V, Singhal M. Aneurysmal right
ventricular outflow in an adult patient with unrepaired tetralogy of
Fallot and an absent pulmonary valve. Journal of Cardiac Surgery.
2021….