Abstract
Residual pulmonary hypertension (PH) in congenital heart defects (CHD) is a condition characterized by persistent or progressive high pressure in the pulmonary artery (PA) following radical correction of CHD. The clinical course and pathophysiology of residual PH after radical CHD correction are similar to idiopathic pulmonary arterial hypertension (PAH). Despite advancements in PAH-specific therapies (endothelin receptor antagonists, soluble guanylate cyclase stimulators, prostanoids), the three-year survival rate of patients does not exceed 68%. Additionally, some patients exhibit refractory disease progression with decompensated right ventricular failure and fatal outcomes.
As a palliative intervention for this patient population, the Potts pulmonary-aortic anastomosis has been proposed. This surgical technique involves creating a connection between the left PA and the descending aorta. The procedure reduces suprasystemic PH, mitigates symptoms of right ventricular failure, and improves patients’ functional status, though it is associated with moderate hypoxemia in the lower body. We present a clinical case of the successful use of the Potts pulmonary-aortic anastomosis in a female patient with residual PH and severe progressive disease refractory to maximal medical therapy.
References
- Chazova I.E., Martynyuk T.V., Valieva Z.S., Azizov V.A., Barbarash O.L., Веселова Т.N. et al. Eurasian clinical guidelines on diagnosis amd treatment of pulmonary hypertension. Eurasian Heart Journal. 2020; 1: 78–12 (in Russ.). DOI: 10.38109/2225-1685-2020-1-78-122
- Gorbachevsky S.V., Shmalts A.A. Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2021; 66 (5): 28–37 (in Russ.). DOI: 10.21508/1027-4065-2021-66-5-28-37
- Chazova I.E., Gorbachevskij S.V., Martynyuk T.V., Shmal’c A.A., Valieva Z.S., Baryshnikova I.Yu. et al. Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension associated with congenital heart defects in adults (2021). Eurasian Heart Journal. 2022; 2: 6–70 (in Russ.). DOI: 10.38109/2225-1685-2022-2-6-70
- Shmalts A.A., Gorbachevsky S.V. Advantages and limitations of initial combination therapy in pulmonary arterial hypertension patients in Russia. Therapeutic Archive. 2020; 92 (12): 80–85 (in Russ.). DOI: 10.26442/00403660.2020.12.000840
- Golukhova E.Z. Report on the scientific and clinical activity of Bakoulev National Medical Research Center for Cardiovascular Surgery for 2023 and development prospects. The Bulletin of Bakoulev Center. Cardiovascular Diseases. 2024; 25 (Special edition). (in Russ.). DOI: 10.24022/1810-0694-2024-25S
- Dovilė J., Virginija R., Gabrielius J., Laurie W.G., Jolien W. et al. Residual pulmonary hypertension more than 20 years after repair of shunt lesions. Medicina (Kaunas). 2020; 56 (6): 297. DOI: 10.3390/medicina56060297
- Gorbachevsky S.V., Pursanov M.G., Shmalts A.A., Dadabaev G.M., Nishonov N.A., Pardaev D.B. et al. Results of stent fenestration of the interatrial septum in patients with idiopathic and similar forms of pulmonary arterial hypertension. Grudnaya i Serdechno-Sosudistaya Khirurgiya. 2019; 61 (2): 100–113 (in Russ.). DOI: 10.24022/0236-2791-2019-61-2-100-113
- Grady R.M. Beyond transplant: roles of atrial septostomy and Potts shunt in pediatric pulmonary hypertension. Pediatr. Pulmonol. 2021; 56
(3): 656–660. DOI: 10.1002/ppul.25049
- Micheletti A., Hislop A.A., Lammers A., Bonhoeffer P., Derrick G., Rees P. et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart. 2006; 92 (7): 969–972. DOI: 10.1136/hrt.2005.077669
- Potts W.J., Smith S., Gibson S. Anastomosis of the aorta to a pulmonary artery; сertain types in congenital heart disease. J. Am. Med. Assoc. 1946; 132: 627–31.
- Blanc J., Vouhé P., Bonnet D. Potts shunt in patients with pulmonary hypertension. N. Engl. J. Med. 2004; 350 (6): 623. DOI: 10.1056/NEJM200402053500623
- Baruteau A.E., Belli E., Boudjemline Y., Laux D., Lévy M., Simonneau G. et al. Palliative Potts shunt for the treatment of children with drug- refractorypulmonary arterial hypertension: updated data from the first 24 patients. Eur. J. Cardiothorac. Surg. 2015; 47: e105–110. DOI: 10.1093/ejcts/ezu445
- Gorbachevskiy S.V., Shmal’ts A.A., Belkina M.V., Grenaderov M.A., Baryshnikova I.Yu., Pursanov M.G. et al. Potts shunt in children with pulmonary hypertension: 7 operations in one clinic and review of world experience. Detskie Bolezni Serdtsa i Sosudov. 2016; 13 (4): 189–198 (in Russ.).
- Grady M.R., Canter M.W., Wan F., Shmalts A.A., Coleman R.D. et al. Pulmonary-to-systemic arterial shunt to treat children with severe pulmonary hypertension. JACC. 2021; 78 (5): 468–477. DOI: 10.1016/j.jacc.2021.05.039
- Latus H., Apitz C., Schmidt D., Jux C., Mueller M. et al. Potts shunt and atrial septostomy in pulmonary hypertension caused by left ventricular disease. Ann. Thorac. Surg. 2013; 96 (1): 317–319. DOI: 10.1016/j.athoracsur.2012.10.069
- Rosenzweig E.B., Ankola A., Krishnan U., Middlesworth W., Bacha E., Bacchetta M. A novel unidirectional-valved shunt approach for end- stage pulmonary arterial hypertension: Early experience in adolescents and adults. Congenit. Mech. Circ. Supp. 2021; 161 (4): 1438–1446. DOI: 10.1016/j.jtcvs.2019.10.149
- Bobhate P., Mohanty S.R., Tailor K., Kadam S., Karande T., Bhavsar K. et al. Potts shunt as an effective palliation for patients with end stage pulmonary arterial hypertension. Ind. Heart J. 2021; 73 (2): 196–204. DOI: 10.1016/j.ihj.2021.01.007
- Zokirov F.K., Chernogrivov I.E., Gorbachevsky S.V. Pulmonary-aortic Potts anastomosis in patients with suprasystemic pulmonary hypertension. Children’s Heart and Vascular Diseases. 2024; 21 (1): 5–16 (in Russ.). DOI: 10.24022/1810-0686-2024-21-1-5-17
- Chernogrivov I.E., Gorbachevskiy S.V., Shmalts A.A., Grenaderov M.A., Belkina M.V., Sabitov A.A. et al. Report on the scientific and practical activities of the Department of Surgical Treatment of Heart Diseases with Progressive Pulmonary Hypertension for 2023. The Bulletin of Bakoulev center. Cardiovascular Diseases. 2024; 25 (6): 587–593 (in Russ.). DOI: 10.24022/1810-0694- 2024-25-6-587-593
- Hopkins W.E., Ochoa L.L., Richardson G.W., Trulock E.P. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J. Heart Lung Transplant. 1996; 15 (1, part 1): 100–105.
About the authors
- Fozilzhon K.U. Zokirov, Cardiovascular Surgeon; ORCID
- Sergey V. Gorbachevskiy, Dr. Med. Sci., Professor, Chief Researcher, Professor of Chair; ORCID
- Igor E. Chernogrivov, Dr. Med. Sci., Head of Department; ORCID
