Pulmonary hypertension surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Richard Channick, M.D.; Assistant Editor(s)-in-Chief: Ralph Matar; Lisa Prior; Ann Slater, R.N.; Rim Halaby, M.D. [2]; José Eduardo Riceto Loyola Junior, M.D.[3]

## Overview

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Patients with severe WHO functional class II or III pulmonary hypertension (PH) refractory to medical therapy are candidates for surgical intervention, such as atrial septostomy or lung transplantation. Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension.

## Surgery

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Surgical intervention such as atrial septostomy or lung transplantation should be considered among patients with pulmonary arterial hypertension (PAH) who fail to improve on optimal therapy or when medical therapy is unavailable.[1]

Failure of clinical improvement among PAH patients with WHO functional class II or III is defined as either:[1]

* A stable and unsatisfactory clinical status, or
* An unstable and deteriorating status

Failure of clinical improvement among PAH patients with WHO functional class IV is defined as either:[1]

* Absence of quick improvement to a WHO functional class III or less, or
* A stable and unsatisfactory clinical status

### Atrial Septostomy

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* Atrial septostomy is a surgical procedure that creates a shunt between the right and left atria.
* It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood (hypoxia). It is best performed in experienced centers.
* Graded balloon dilatation of atrial septostomy is the recommended type of atrial septostomy.[2]
* Atrial septostomy might be considered among PH patients with WHO functional class IV and right heart failure refractory to therapy.[3]
* Atrial septostomy should also be considered among PH patients who have Eisenmenger's syndrome, idiopathic PAH, and those awaiting lung transplantation.[1]

### Lung Transplantation

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* Lung transplantation is considered in the treatment of patients with idiopathic PH, PH associated with congenital heart disease, or pulmonary veno-occlusive disease (PVOD) who fail to improve on optimal medical therapy.
* Combined lung and heart transplantation might be considered in selected patients.[1]
* According to the Registry of the International Society for Heart and Lung Transplantation, the survival rates following lung transplantation are 61%, 49%, and 25 % at 3, 5, and 10 years respectively.[4]
* If patients present with rapidly progressing disease, pulmonary transplantation should be considered earlier.[5]

### Pulmonary Thromboendarterectomy

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* Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for the treatment of chronic thromboembolic pulmonary hypertension.
* It is the surgical removal of an organized thrombus along with the lining of the pulmonary artery.
* PTE is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
* Treatment for hypoxic and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.

## References

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1. ↑ 1.0 1.1 1.2 1.3 1.4 Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA; et al. (2009). "Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)". Eur Heart J. 30 (20): 2493–537. doi:10.1093/eurheartj/ehp297. PMID 19713419.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
2. ↑ Sandoval J, Gaspar J, Pulido T, Bautista E, Martínez-Guerra ML, Zeballos M; et al. (1998). "Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment". J Am Coll Cardiol. 32 (2): 297–304. PMID 9708453.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
3. ↑ Kurzyna M, Dabrowski M, Bielecki D, Fijalkowska A, Pruszczyk P, Opolski G; et al. (2007). "Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension". Chest. 131 (4): 977–83. doi:10.1378/chest.06-1227. PMID 17426198.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
4. ↑ Trulock EP, Edwards LB, Taylor DO, Boucek MM, Keck BM, Hertz MI; et al. (2006). "Registry of the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart-lung transplantation report--2006". J Heart Lung Transplant. 25 (8): 880–92. doi:10.1016/j.healun.2006.06.001. PMID 16890108.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
5. ↑ Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check `|pmid=` value (help).

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