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Pediatric Cardiology

, Volume 40, Issue 4, pp 841–847 | Cite as

Relationship Between Pulmonary Arterial Resistance and Compliance in Patients with Down Syndrome

  • Yuka Iwaya
  • Jun MuneuchiEmail author
  • Yuka Inoue
  • Mamie Watanabe
  • Seigo Okada
  • Yoshie Ochiai
Original Article
  • 132 Downloads

Abstract

This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)–compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6–4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62–3.0) vs. 2.50 (1.86–3.31) mL/mmHg/m2, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.

Keywords

Pulmonary arterial hypertension Pulmonary arterial capacitance Trisomy 21 Congenital heart disease Chromosomal anomaly 

Abbreviations

ANCOVA

Analysis of covariance

Cp

Pulmonary arterial compliance

DS

Down syndrome

HOT

Home oxygen therapy

lnRp

Logarithm transformation of Rp

lnCp

Logarithm transformation of Cp

PAH

Pulmonary arterial hypertension

PAP

Pulmonary arterial pressure

Qp

Pulmonary blood flow

Qs

Systemic blood flow

Qp/Qs

The ratio of pulmonary to systemic blood flow

Rp

Pulmonary arterial resistance

Notes

Acknowledgements

We gratefully thank M.Miura, Clinical researcher at Tokyo Metropolitan Children’s Medical Center (Tokyo, Japan), for advising about statistical analysis for the manuscript.

Compliance with Ethical Standards

Conflict of interest

The authors have no conflicts of interest to declare.

Ethical Approval

All procedures performed in this study involving human participants were in accordance with the Institutional Ethics Committee of Kyushu Hospital, Japan Community Healthcare Organization (approval number 463) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all patients or their guardians included in the study.

References

  1. 1.
    Chi TPL, Krovetz J (1975) The pulmonary vascular bed in children with Down syndrome. J Pediatr 86:533–538CrossRefGoogle Scholar
  2. 2.
    King P, Tulloh R (2011) Management of pulmonary hypertension and Down syndrome. Int J Clin Pract Suppl 174:8–13CrossRefGoogle Scholar
  3. 3.
    Cooney TP, Thurlbeck WM (1982) Pulmonary hypoplasia in Down’s syndrome. N Engl J Med 307:1170–1173CrossRefGoogle Scholar
  4. 4.
    Yamaki S, Horiuchi T, Sekino Y (1983) Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with the Down syndrome. Am J Cardiol 51:1502–1506CrossRefGoogle Scholar
  5. 5.
    de Miguel-Díez J, Villa-Asensi JR, Alvarez-Sala JL (2003) Prevalence of sleep-disordered breathing in children with Down syndrome: polygraphic findings in 108 children. Sleep 26:1006–1009CrossRefGoogle Scholar
  6. 6.
    Cua CL, Rogers LK, Chicoine LG, Augustine M, Jin Y, Nash PL, Nelin LD (2011) Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine. Eur J Pediatr 170:859–863CrossRefGoogle Scholar
  7. 7.
    Fukushima H, Kosaki K, Sato R, Yagihashi T, Gatayama R, Kodo K, Hayashi T, Nakazawa M, Tsuchihashi T, Maeda J, Kojima Y, Yamagishi H, Takahashi T (2010) Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: an imbalance in biosynthesis of thromboxane A2 and prostacyclin. Am J Med Genet A 152:1919–1924CrossRefGoogle Scholar
  8. 8.
    Sagawa K, Lie RK, Schaefer J (1990) Translation of Otto Frank’s paper “Die Grundform des Arteriellen Pulses”. Zeitschrift für Biologie 37:483–526 (1899). J Mol Cell Cardiol 22:253–254.CrossRefGoogle Scholar
  9. 9.
    Muneuchi J, Nagatomo Y, Watanabe M, Joo K, Onzuka T, Ochiai Y, Joo K (2016) Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease. J Thorac Cardiovasc Surg 152:507–513CrossRefGoogle Scholar
  10. 10.
    Hoashi T, Hirahara N, Murakami A, Hirata Y, Ichikawa H, Kobayashi J, Takamoto S (2017) Current surgical outcomes of congenital heart surgery for patients with Down Syndrome in Japan. Circ J 82:403–408CrossRefGoogle Scholar
  11. 11.
    Kumar B, Puri GD, Manoj R, Gupta K, Shyam KS (2011) Severe pulmonary artery hypertension following intracardiac repair of tetralogy of Fallot: an unusual finding. Pulm Circ 11:115–118CrossRefGoogle Scholar
  12. 12.
    Muneuchi J, Oda S, Shimizu D (2017) Rapidly progressive pulmonary veno-occulusive disease in an infant with Down syndrome. Cardiol Young 27:1402–1405CrossRefGoogle Scholar
  13. 13.
    Takatsuki S, Nakayama T, Ikehara S, Matsuura H, Ivy DD, Saji T (2017) Pulmonary arterial capacitance index is a strong predictor for adverse outcome in children with idiopathic and heritable pulmonary arterial hypertension. J Pediatr 180:75–79CrossRefGoogle Scholar
  14. 14.
    Shott SR, Amin R, Chini B, Heubi C, Hotze S, Akers R (2006) Obstructive sleep apnea: should all children with Down syndrome be tested? Arch Otolaryngol Head Neck Surg 132:432–436CrossRefGoogle Scholar
  15. 15.
    Jacobs IN, Teague WG, Bland JW Jr (1997) Pulmonary vascular complications of chronic airway obstruction in children. Arch Otolaryngol Head Neck Surg 123:700–704CrossRefGoogle Scholar
  16. 16.
    Hawkins A, Langton-Hewer S, Henderson J, Tulloh RM (2011) Management of pulmonary hypertension in Down syndrome. Eur J Pediatr 170:915–921CrossRefGoogle Scholar
  17. 17.
    Saji T (2014) Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int 56:297–303CrossRefGoogle Scholar
  18. 18.
    Suzuki K, Yamaki S, Mimori S, Murakami Y, Mori K, Takahashi Y, Kikuchi T (2000) Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect. Am J Cardiol 86:434–437CrossRefGoogle Scholar
  19. 19.
    Galambos C, Minic AD, Bush D, Nguyen D, Dodson B, Seedorf G, Abman SH (2016) Increased lung expression of anti-angiogenic factors in down syndrome: potential role in abnormal lung vascular growth and the risk for pulmonary hypertension. PLoS ONE 11:e0159005CrossRefGoogle Scholar
  20. 20.
    Fukushima H, Kosaki K, Sato R, Yagihashi T, Gatayama R, Kodo K, Hayashi T, Nakazawa M, Tsuchihashi T, Maeda J, Kojima Y, Yamagishi H, Takahashi T (2010) Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: an imbalance in biosynthesis of thromboxane A2 and prostacyclin. Am J Med Genet A 152A:1919–1924CrossRefGoogle Scholar
  21. 21.
    Kageyama K, Hashimoto S, Nakajima Y, Shime N, Hashimoto S (2007) The change of plasma endothelin-1 levels before and after surgery with or without Down syndrome. Paediatr Anaesth 17:1071–1077Google Scholar
  22. 22.
    Okada S, Muneuchi J, Nagatomo Y, Watanabe M, Iida C, Shirouzu H, Matsuoka R, Joo K (2017) Pulmonary arterial resistance and compliance in preterm infants. Int J Cardiol 244:265–270CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Pediatric Cardiology, Department of Pediatrics, Japan Community Healthcare OrganizationKyushu HospitalKitakyushuJapan
  2. 2.Division of Pediatric Cardiology, Department of Cardiovascular Surgery, Japan Community Healthcare OrganizationKyushu HospitalKitakyushuJapan

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