Heart & Lung 43 (2014) 367e370

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Primary pulmonary vein stenosis in a premature infant without bronchopulmonary dysplasia: A case report Lijian Xie, MD, Tingting Xiao, MD, Jie Shen, MD * Department of Cardiology, Shanghai Children’s Hospital, Shanghai Jiao Tong University, West Beijing Road 1400-24, Shanghai 200040, PR China

a r t i c l e i n f o

a b s t r a c t

Article history: Received 27 January 2014 Received in revised form 13 March 2014 Accepted 18 April 2014

Primary pulmonary vein stenosis (PVS) presenting in childhood is uncommon and is related to premature with bronchopulmonary dysplasia (BPD). Here we present a premature infant with primary PVS and without BPD. In our case, a 19-month-old girl was diagnosed with PVS, atrial septal defect and patent arterial duct by echocardiography and selective pulmonary artery angiography. Interestingly in the first 2 month after birth, there was no clue of PVS by bed echocardiography in the patient. It is important to focus on the pulmonary vein blood velocity in premature infants and very low birth weight infants even without BPD. Ó 2014 Elsevier Inc. All rights reserved.

Keywords: Pulmonary vein Stenosis Premature

Introduction Pulmonary vein stenosis (PVS) is a rare condition characterized by the narrowing of one or more pulmonary veins at their left atrial opening. Primary PVS is most frequently associated with cardiac malformations such as anomalous pulmonary venous return.1 It can lead to worsening pulmonary hypertension and cardiac failure in the pediatric population. Neither surgery nor transcatheter interventions have yielded satisfactory long term results.2 Recent years have seen significantly improved perinatal survival for extremely premature infants, resulting in a number of infants and children suffering with its common pulmonary sequela, bronchopulmonary dysplasia (BPD).3 Recent case and epidemiologic studies have identified a hitherto-unappreciated association between the PVS and BPD.4e6 Here we report a premature infant with primary PVS and without BPD at our institution. Case report A 19-month-old girl was admitted for heart surgery with a secundum atrial septal defect (ASD) and patent arterial duct (PDA). The patient was the mother’s third child from her second pregnancy, the younger of twins and born at 36 weeks gestation. She was delivered by cesarean section and her birth weight was only 1.09 kg. Her Apgar scores were 7 and 8 at 1 and 9 min, and she was given oxygen, was intubated and received parenteral nutrition in the neonatal

* Corresponding author. Tel.: þ86 13918874909. E-mail address: [email protected] (J. Shen). 0147-9563/$ e see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.hrtlng.2014.04.018

intensive care unit. She had mild perinatal indirect hyperbilirubinemia managed by phototherapy and, as demonstrated on bed echocardiography, an atrial septal defect, a ventricular septal defect, a patent arterial duct and pulmonary hypertension. Further more, the color Doppler and pulse wave Doppler demonstrated normal pulmonary vein velocity (Fig. 1). Then she was received diuretic hydragogue to decrease the cardiac load. She developed neonatal sepsis 3 weeks later and received injected antibiotics and gamma globulin. After 2 months, her body weight was 1.90 kg and she was discharged from hospital. The patient was not received surfactant and was not required positive pressure ventilation in NICU. Her vital signs were pulse 118/min, respiratory rate 26/min, weight 6 kg and oxygen saturation 98% in room air. Physical examination revealed a soft upper left parasternal systolic murmur with a prominent pulmonary second heart sound. Electrocardiography showed sinus rhythm. Chest radiography showed a slightly enlarged heart shadow and lung field congestion. There was no evidence of abnormal branch tract or lung development on computed tomography of the chest. Transthoracic echocardiography showed atrial septal defect (secundum type, Ø 10 mm), patent arterial duct (Ø 1.0 mm), abnormal increased right upper pulmonary vein blood velocity (193 cm/s), and pulmonary hypertension as estimated by the tricuspid reflex (DP 56 mm Hg) (Fig. 2). Heart catheterization demonstrated a pulmonary capillary wedge pressure of 19/ 10 mm Hg (mean 11), main pulmonary artery pressure 70/38 mm Hg (mean 54), right ventricular pressure 87/0 mm Hg (mean 3), and left ventricular pressure 126/1 mm Hg (mean 16). Selective pulmonary vein angiography showed stenosis of four pulmonary veins at the venoatrial junction (Fig. 3).

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Fig. 1. (A) The two atrium view showed atrial septal defect (right to left shunt) with color Doppler. (B) The two atrium view showed atrial septal defect (left to right shunt) with color Doppler. (C) The four chamber view showed normal blood reflux in right upper pulmonary vein with color Doppler. (D) Right upper pulmonary vein blood flow velocity demonstrated by continuous wave Doppler. (E) Left upper pulmonary vein blood flow velocity demonstrated by continuous wave Doppler. (F) Left down pulmonary vein blood flow velocity demonstrated by continuous wave Doppler.

Discussion Primary PVS is a rare anomaly in association with anomalous pulmonary venous return. However, there is recent evidence of increased risk of primary PVS in premature infants with BPD. A recent clinicopathologic population study by Drossner and colleagues identified prematurity as being highly associated with PVS (odds ratio: 10.2; 95% confidence interval: 4.7e22.6; P < 0.001); of 26 cases of PVS, 11 (42%) had comorbid BPD.4 The pathogenetic hierarchy of PVS is not clear. Drossner et al have speculated that

many of the causal mechanisms of BPD, especially its small vascular component, might contribute to the endovascular proliferative stenosis observed in PVS, while PVS itself may contribute to the syndrome of BPD through its induction of vascular congestion, pulmonary edema, and increased right-sided heart pressures.4 Sadr et al7 postulated that abnormal cytokine production, flow abnormalities and post-surgical stress may be triggers of the process. The autopsy of an premature infant with PVS and BPD showed fibrous ridge in pulmonary vein obstructing atrial ostia and microscopically the lungs showed patchy edema, severe capillary congestion,

Fig. 2. (A) Shunt from the left atrium to the right atrium, indicating atrial septal defect. (B) A small patent arterial duct shunt on a short axis view. (C) Right upper pulmonary vein flow was brightly colored, indicating a high blood flow velocity. (D) Pulmonary vein blood flow velocity (193 cm/s) demonstrated by continuous wave Doppler.

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Fig. 3. Selective pulmonary vein angiography in AP position demonstrated stenoses (arrow) of right upper, right down, left upper and left down pulmonary veins at the venoatrial junction. The angiography movie also showed the pulmonary vein reflux was jetted in atrium.

dilatation of pulmonary veins associated with congestion, and focal thrombosis secondary to venous obstruction.8 A consanguineous Turkish family with four siblings with primary PVS associated with prenatal lymphatic anomalies has been reported.6 A genome-wide linkage analysis of this family was the first to identify a locus (chromosome 2q35-2q36.1) for primary pulmonary vein stenosis. SGPP2, which encodes sphingosine-1-phosphate phosphatase 2 (involved in controlling the proliferation and migration of various cell types, including vascular smooth cells, cardiomyocytes and endothelial cells) is a promising candidate gene.9 In our case, a very low birth weight premature infant developed PVS after birth, although there was no evidence of BPD. Interestingly in the first 2 month after birth, there was no sign of PVS in symptom and no clue found by bed echocardiography. PVS was found until 19 month old. It exhibited slight symptoms and it is difficult to diagnose by echocardiography at starting time after birth. Also, our case confirmed PVS is related with premature and low birth weight. Acquired PVS after radiofrequency ablation for atrial fibrillation in adults is due to periadventitial inflammation and/or collagen deposition secondary to thermal injury of the pulmonary vein musculature.10,11 We think primary PVS may be a developing progress similar with acquired PVS in histopathology according to our case. Conclusion Primary PVS in infant is very rare. However, with the progress of perinatal survival for premature infants and very low birth weight infants, PVS is becoming the potential vital factor of prognosis. Moreover, PVS is easily omitted in infants in NICU only with bed

echocardiography. It is important to focus on the pulmonary vein blood velocity in premature infants and very low birth weight infants even without BPD. Acknowledgment The authors are very grateful to Dr Zhao jian, Shanghai Children’s Hospital, for collecting the clinical data. Financial support: The article received no specific grant from any funding agency, commercial or no-for-profit sectors. Conflicts of interest: None. Ethical standards: The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the Ethics Committee of Shanghai Children’s Hospital. References 1. Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation. 2007;115:103e108. 2. Peng LF, Lock JE, Nugent AW, Jenkins KJ, McElhinney DB. Comparison of conventional and cutting balloon angioplasty for congenital and postoperative pulmonary vein stenosis in infants and young children. Catheter Cardiovasc Interv. 2010;75:1084e1090. 3. Kinsella JP, Greenough A, Abman SH. Bronchopulmonary dysplasia. Lancet. 2006;367:1421e1431. 4. Drossner DM, Kim DW, Maher KO, Mahle WT. Pulmonary vein stenosis: prematurity and associated conditions. Pediatrics. 2008;122:e656ee661. 5. Holt DB, Moller JH, Larson S, Johnson MC. Primary pulmonary vein stenosis. Am J Cardiol. 2007;99:568e572. 6. Chakrabarti S, Mittal R, Gnanapragasam JP, Martin RP. Acquired stenosis of normally connected pulmonary veins. Cardiol Young. 2007;17:322e327.

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7. Sadr IM, Tan PE, Kieran MW, Jenkins KJ. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol. 2000;86:577e579. A10. 8. Smith SC, Rabah R. Pulmonary venous stenosis in a premature infant with bronchopulmonary dysplasia: clinical and autopsy findings of these newly associated entities. Pediatr Dev Pathol. 2012;15:160e164. 9. van de Laar I, Wessels M, Frohn-Mulder I, et al. First locus for primary pulmonary vein stenosis maps to chromosome 2q. Eur Heart J. 2009;30(20):2485e2492.

10. Holmes Jr DR, Monahan KH, Packer D. Pulmonary vein stenosis complicating ablation for atrial fibrillation: clinical spectrum and interventional considerations. JACC Cardiovasc Interv. 2009;2:267e276. 11. Neumann T, Kuniss M, Conradi G, et al. Pulmonary vein stenting for the treatment of acquired severe pulmonary vein stenosis after pulmonary vein isolation: clinical implications after long-term follow-up of 4 years. J Cardiovasc Electrophysiol. 2009;20:251e257.

Primary pulmonary vein stenosis in a premature infant without bronchopulmonary dysplasia: a case report.

Primary pulmonary vein stenosis (PVS) presenting in childhood is uncommon and is related to premature with bronchopulmonary dysplasia (BPD). Here we p...
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