Pulmonary Arterial Hypertension (PAH)

Significant pharmacologic advances have been made in the treatment of pulmonary arterial hypertension (PAH). However, patients with PAH that is refractory to pharmacologic treatment may consider surgical options, given the significant effect of this disease on the right ventricle of the heart.1

Atrial Septostomy

Atrial septostomy (AS) is an open-heart surgery that may be used to control the signs and symptoms of PAH.2 Research has shown that AS can result in clinical improvements in the cardiac index and exercise tolerance among patients with PAH that does not respond to pharmacologic treatment.3 

In this procedure, placement of a cardiac catheter through an opening between the left and right atria of the heart creates a right-to-left shunt.4 The 2 main techniques used for AS are stepwise balloon dilation and blade balloon AS.1 AS reduces the pressure in the right side of the heart, allowing the heart to work more efficiently and improving blood flow to the lungs.4 

Despite its benefits, AS can be lethal; the rate of immediate mortality is approximately 13% to 16%. Contraindications to AS include oxygen saturation below 90%, low cardiac output, and mean right atrial pressure above 20 mm Hg.1 Arrhythmias may develop as complications of this procedure.2

Other right-to-left shunting strategies include use of an atrial flow regulator and a Potts shunt or modifications of a Potts shunt.1

Implanted Right Ventricular Assist Devices

PAH can cause right ventricular failure. Mechanical support for the heart with right ventricular assist devices can reduce the stroke volume, improving septal motion and left ventricular diastolic filling.1 Right ventricular assist devices can be implanted with either right ventricular sourcing and right atrial sourcing.1

Pulmonary Endarterectomy

Pulmonary endarterectomy (PEA) is used to relieve pulmonary arterial obstruction in patients with chronic thromboembolic pulmonary hypertension (CTEPH).4,5 Clot removal reduces the pulmonary vascular resistance and alleviates right ventricular dysfunction.5 

PEA is a demanding procedure that must be performed by surgeons with specialized training. However, it can relieve symptoms and prolong survival in patients with CTEPH, with immediate effects.5,6 The procedure entails cardiopulmonary bypass and deep hypothermic circulatory arrest. Reperfusion lung injury and persistent pulmonary hypertension are a few of the complications associated with PEA.5

Balloon Pulmonary Angioplasty

Not all patients with CTEPH are candidates for PEA, and many who undergo this procedure may experience residual or recurrent pulmonary hypertension; therefore, potential alternatives have been developed.6 Balloon pulmonary angioplasty (BPA), also known as percutaneous transluminal pulmonary angioplasty, may benefit patients with CTEPH; research indicates that BPA can improve cardiac function and exercise tolerance.6 

In BPA, a balloon is used to dilate and open stenotic or blocked pulmonary vessels. The technique is performed under angiographic guidance; therefore BPA is contraindicated for patients who are allergic to iodine because contrast agents must be used.6 Complications associated with the procedure include lung injury and renal dysfunction.6

Extracorporeal Cardiopulmonary Support

Extracorporeal membrane oxygenation (ECMO) may be used in patients with end-stage PAH and in those recovering from a transplant procedure or PEA.7 

The 2 types of ECMO are VV-ECMO, used for respiratory failure, and veno-arterial ECMO (VA-ECMO), which has been determined to be appropriate for patients with PAH.1 During the surgical procedure to implement ECMO, the target vessels are visualized and cannulated; typically, a venous cannula is placed within the right atrium and superior vena cava.1 

ECMO can be life-sustaining and may be used as a bridge to transplant. ECMO can help to reverse complications that may occur as a patient awaits surgery, such as liver failure and pre-renal failure, by improving systemic circulation and oxygenation.1

Lung Transplant

Lung and heart transplants are reserved for patients who do not respond to any other type of treatment.1 The procedure most frequently performed in patients with PAH is the double lung transplant.1,8 A heart-lung transplant can be used for patients with severe PAH and irreversible ventricular heart failure.1 The overall survival rate of patients who have received a lung transplant can be as high as 50% after 7 years; the rate is higher in those who have received a double transplant.1 Patients with idiopathic PAH account for approximately 5% of all those who undergo a double lung transplant. Lung transplants are performed more frequently in patients with PAH who have another condition, such as chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, or connective tissue disease.9 

Following a transplant, the pressure in the pulmonary arteries decreases, and the heart can recover. Patients must receive immunosuppressive medication, which may increase the risk for infection or cancer.1


1. Stącel T, Latos M, Urlik M, et al. Interventional and surgical treatments for pulmonary arterial hypertension. J Clin Med. 2021;10(15):3326. doi:10.3390/jcm10153326

2. Pulmonary hypertension diagnosis and treatment. Mayo Clinic. Accessed March 14, 2022.

3. Keogh AM, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S67-S77. doi:10.1016/j.jacc.2009.04.016

4. Treatment pulmonary hypertension. NHS. Accessed March 14, 2022

5. Jenkins D. Pulmonary endarterectomy: the potentially curative treatment for patients with chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2015;24(136):263-271. doi:10.1183/16000617.00000815

6. Jin Q, Zhao ZH, Luo Q, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: state of the art. World J Clin Cases. 2020;8(13):2679-2702. doi:10.12998/wjcc.v8.i13.2679

7. Fadel E, Mercier O, Mussot S, et al. Traitement chirurgical de l’hypertension artérielle pulmonaire. Surgical treatment of pulmonary arterial hypertension [in French]. Rev Mal Respir. 2011;28(2):139-151. doi:10.1016/j.rmr.2010.09.024

8. Transplant and pulmonary hypertension. Pulmonary Hypertension Association. Accessed March 14, 2022

9. Answers About Lung Transplantation for Pulmonary Hypertension. Pulmonary Hypertension Association. Accessed March 14, 2022

Reviewed by Harshi Dhingra, MD, on 3/15/2022.