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Case Reports
. 2020 Nov 10;10(4):2045894020954310.
doi: 10.1177/2045894020954310. eCollection 2020 Oct-Dec.

Peripheral pulmonary stenosis with Noonan syndrome treated by balloon pulmonary angioplasty

Seien Ko  1 , Jin Komuro  1 , Yoshinori Katsumata  1 , Yasuyuki Shiraishi  1 , Takashi Kawakami  1 , Yoshitake Yamada  2 , Shinsuke Yuasa  1 , Takashi Kohno  1   3 , Kenjiro Kosaki  4 , Keiichi Fukuda  1
Affiliations
Case Reports

Peripheral pulmonary stenosis with Noonan syndrome treated by balloon pulmonary angioplasty

Seien Ko et al. Pulm Circ. .

Abstract

Noonan syndrome is known to have various cardiovascular defects, which include pulmonary artery stenosis. Pulmonary artery stenosis is characterized by obstruction of pulmonary artery blood flow that can cause elevated pulmonary artery pressure and ventilation-perfusion inequality, which can cause dyspnea on exertion and eventually, heart failure. Although the etiology of pulmonary artery stenosis related to congenital diseases is still unknown, balloon pulmonary angioplasty has being reported to be effective to selected patients with Alagille and Williams syndromes, but not from Noonan syndrome despite of modest prevalence of pulmonary artery stenosis. Here, we report the first Noonan syndrome patient with pulmonary artery stenosis who underwent successful balloon pulmonary angioplasty. The strategy used in balloon pulmonary angioplasty was planned with careful morphologic evaluation by computed tomographic angiography, and performed with scoring balloons in a graded approach with multiple sessions. After balloon pulmonary angioplasty, we confirmed maintained dilation of lesions and symptom alleviation, suggesting that balloon pulmonary angioplasty can be performed safely on pulmonary artery stenosis in a Noonan syndrome patient.

Keywords: catheter intervention; pulmonary hypertension; ventilation perfusion ratio inequality.

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Conflict of interest statement

Conflict of interest: The author(s) declare that there is no conflict of interest.

Figures

Fig. 1.
Fig. 1.
(a) Oblique coronal, (b) oblique axial Image of maximal intensity projection computed tomography angiography (CTA) focusing on the strictures (arrows) in the left pulmonary artery branches. (c) Images of 81mKr ventilation scintigraphy are shown in upper row. Images of 99mTc-macro aggregated albumin (MAA) lung perfusion scintigraphy is shown in the lower row. There is significant decrease of 99mTc-MAA uptake. LAO: left anterior oblique. RAO: right anterior oblique. RPO: right posterior oblique. LPO: left posterior oblique. (d)–(i) Pulmonary artery angiographs (PAGs) of the patient. (d) PAG of right and (e) left pulmonary arteries before balloon pulmonary angioplasty (BPA). (f) Selective PAG of the left lower branches and target lesion of BPA (arrow). (g) Balloon (arrow) dilating the lesion. (h) Improved blood flow distal to the lesion (arrow) right after BPA. (i) PAG at 18 months follow-up showing improved dilation (arrow).
See this image and copyright information in PMC

References

    1. Mainwaring RD, Hanley FL. Surgical techniques for repair of peripheral pulmonary artery stenosis. Semin Thorac Cardiovasc Surg 2017; 29(2): 198–205. - PubMed
    1. Sznajer Y, Keren B, Baumann C, et al. The spectrum of cardiac anomalies in Noonan syndrome as a result of mutations in the PTPN11 gene. Pediatrics 2007; 119(6): e1325–e1331. - PubMed
    1. Ishizawa A, Oho S, Dodo H, et al. Cardiovascular abnormalities in Noonan syndrome: the clinical findings and treatments. Acta Paediatr Jpn 1996; 38(1): 84–90. - PubMed
    1. Tonelli AR, Ahmed M, Hamed F, et al. Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults. Pulm Circ 2015; 5(1): 204–210. - PMC - PubMed
    1. Gay BB, Franch RH, Shuford WH, et al. The roentgenologic features of single and multiple coarctations of the pulmonary artery and branches. Am J Roentgenol 1963; 90(1): 599–613. - PubMed

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