pulmonary%20arterial%20hypertension
PULMONARY ARTERIAL HYPERTENSION
Pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately leading to right heart failure.
It is a part of the spectrum of pulmonary hypertension, which is hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest.
Typical symptoms include progressive dyspnea on exertion, palpitations, fatigue, weakness, angina, syncope and abdominal distention.

Surgical Intervention

Balloon Atrial Septostomy (BAS)

  • Several experimental & clinical observations suggest that an atrial defect may be of benefit in the setting of pulmonary hypertension (PH) as it would allow right to left shunting which increases systemic output
    • This would increase systemic arterial oxygen saturation
    • It also decompresses the right atrium & ventricle therefore decreasing signs & symptoms of right heart failure
  • At present, indications for BAS are:
    • Patients who are in World Health Organization functional class (WHO FC) IV with severe syncope &/or right heart failure despite all available medical treatments
    • Palliative bridge to lung transplantation
    • Sole treatment modality when other options are not available
    • Considered in pulmonary arterial hypertension (PAH) associated with surgically corrected congenital hear disease (CHD), connective tissue disease, distal chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary veno-occlusive disease & pulmonary capillary hemangiomatosis
    • May be considered before severe hemodynamic compromise & end-organ dysfunction occur
  • Should be performed only at institutions with significant procedural & clinical experience
  • Avoided in patients with a baseline mean right atrial pressure (RAP) of 20 mmHg & O2 saturation at rest of <85% on room air

Transplantation

  • There is no agreement on the optimal type of transplantation for patients with PAH, whether single lung, double lung or combined heart & lung
  • Indicated in PAH patients when they have an unacceptable response to PAH therapies seen in FC III or IV
  • Due to lack of effective medical therapeutic regimen, patients with pulmonary veno-occlusive disease & pulmonary capillary hemangiomatosis should be candidates for transplantation at diagnosis
  • Survival rates in PAH patients undergoing transplant: 66% at 1 year, 57% at 3 years, 47% at 5 years & 27% at 10 years
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