Complex treatment of recurrent pulmonary vein stenosis

Y. Mykychak

doi:10.30702/ujcvs/18.33/23(094-096)

Y. Mykychak Ukrainian Children’s Cardiac Center (Kyiv)

DOI: https://doi.org/10.30702/ujcvs/18.33/23(094-096)

Keywords: pulmonary vein stenosis, pulmonary hypertension, totally anomalous pulmonary venous connections

Abstract

Pulmonary vein stenosis (PVS) in children is a rare heart disease characterized by high recurrence and mortality rates.

Objective. We describe a case of recurrent PVS, which was treated surgically and subsequently with repeated balloon angioplasties.

Materials and methods. Echocardiogram showed total anomalous pulmonary venous connection, mixed form. A CT scan of the chest confirmed the diagnosis. Month later after initial surgery angiography detected the presence of stenosis in all pulmonary veins.

Results. Currently patient has moderate residual stenosis in each PV. His prognosis is considered bleak due to continuous requirement in aggressive interventions in order at least temporarily resolve pulmonary vein stenosis.

Conclusions. Pulmonary vein stenosis still bears high recurrence, reintervention and mortality rates. Rigorous surveillance and combination of surgical and interventional treatment is warranted in this subset of patients to maintain quality of life.

References

1. REYE RDK. Congenital stenosis of the pulmonary veins in their extrapulmonary course. Med. J. Aust. 1951; 1(22):801-802.

2. Lacour-Gayet F, Lopez L, Davidson J, da Cruz EM. Pulmonary Vein Stenosis. In Da Cruz EM, Ivy D, Jaggers J (eds): Pediatr. Congenit. Cardiol. Card. Surg. Intensive Care, London: Springer London, 2014:1905-1918.

3. Mueller C, Dave H, Pretre R. Primary correction of total anomalous pulmonary venous return with a modified sutureless technique. Eur J Cardiothorac Surg 2012;43:635-40.

4. Kalfa DK, Belli E, Bacha E, Lambert V, di Carlo D, Kostolny M, et al. Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era. J Thorac Cardiovasc Surg. 2018;156:276-86.

5. Quinonez LG, Gauvreau K, Borisuk M, Ireland C, Marshall AM, Mayer JE, et al. Outcomes of surgery for young children with multivessel pulmonary vein stenosis. J Thorac Cardiovasc Surg. 2015;150:911-7.

6. Rachel D. Vanderlaan and Christopher A. Sutureless repair and postoperative residual stenosis: ‘‘Never leave with a lesion”. J Thorac Cardiovasc Surg 2018;156:287-8.