Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension

Iwaya, Yuki; Muneuchi, Jun; Watanabe, Mamie; Sugitani, Yuichiro; Ochiai, Yoshie

doi:10.1007/s00246-020-02400-4

Cited by 2 publications

(4 citation statements)

References 21 publications

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“…Our previous study has shown that low Cp is associated with postoperative pulmonary hypertension and oxygen requirement in T21 individuals (Iwaya et al, 2020). Therefore, the preoperative assessment by Rp and Cp are essential in patients with genetic disorder who have congenital heart disease and pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

“…When assessing the pulmonary circulation, it is important to consider both pulmonary vascular resistance (Rp) and compliance (Cp), which refers the two‐element Windkessel models (Ghio et al, 2015). The measurement of Cp has recently gained interests in patients with pulmonary hypertension associated with congenital heart disease (Muneuchi et al, 2016, Iwaya et al, 2020, Doi et al, 2021), because Cp consistently predicts mortality and morbidity compared to Rp in patients with different causes of pulmonary hypertension, including idiopathic/hereditary pulmonary arterial hypertension or pulmonary hypertension associated with left heart dysfunction (Pellegrini et al, 2014; Takatsuki et al, 2017). Individuals with trisomy 21 (T21) with congenital heart disease and pulmonary hypertension have been shown to have a unique pulmonary vascular condition characterized by decreased Cp compared to individuals without chromosomal anomalies (Iwaya et al, 2019), which reminds us of the hypothesis that T18 individuals with congenital heart disease will have the similar pulmonary circulation.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary vascular resistance and compliance in individuals with trisomy 18

Hatai

Muneuchi

Sugitani

et al. 2021

American J of Med Genetics Pt A

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Individuals with trisomy 18 (T18) usually have congenital heart disease, often with pulmonary hypertension, which is associated with poor outcomes. This study aimed to explore the characteristics of pulmonary circulation including pulmonary vascular resistance (Rp) and compliance (Cp) among them. We retrospectively reviewed cardiac catheterization data in subjects with T18, trisomy 21 (T21), and without chromosomal anomaly (control group) who were referred due to heart failure associated with ventricular septal defect between 2000 and 2020. Pulmonary hemodynamic parameters including Rp and Cp were compared between these groups. We studied 20 subjects with T18, 88 subjects with T21, and 240 control subjects. There was no significant difference in age (T18: 4.6 [3.0–6. 9] vs. T21: 2.8 [1.9–4.0] vs. control: 2.9 [1.6–3.2] months, p = 0.06) and mean pulmonary arterial pressure (T18: 41 [33–49] vs. T21: 35 [30–41] vs. control: 36 [28–43] mmHg, p = 0.121) between the groups. The pulmonary to systemic blood flow ratio (Qp/Qs) (p = 0.983), Rp (p = 0.449), and Cp (p = 0.195) did not differ between T18 and control groups. However, Qp/Qs and Cp in T18 group were significantly greater than that in T21 group (T18: Qp/Qs: 3.4 [2.3–5.2] vs. T: 21 2.3 [1.7–3.7], p = 0.001. Cp: 3.5 [2.3–5.5] vs. 2.3 [1.6–3.1] mmHg/mL/m2, p = 0.007), while Rp was identical between the groups (T18: 2.0 [1.6–3.3] vs. T21: 2.3 [1.7–3.7], p = 0.386). The pulmonary circulation in T18 subjects differed from that observed in T21 subjects, and identical to that observed in control subjects. Pulmonary hypertension is expected to be normalized after reasonable corrective surgery in T18 patients with congenital heart disease.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pulmonary vascular resistance and compliance in individuals with trisomy 18

Hatai

Muneuchi

Sugitani

et al. 2021

American J of Med Genetics Pt A

Self Cite

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“…Second, individuals with trisomy 21, who often accompany CHD, have a unique pulmonary vasculature characterized by low Cp compared to subjects without chromosomal anomalies (25,27). Although individuals with trisomy 21 have an increased risk of developing pulmonary arterial hypertension and require careful management during the perioperative period (52), pulmonary hypertension associated with trisomy 21 is caused by heterogeneous factors, including left-to-right shunt CHD, abnormal pulmonary vasculature growth, hypoxic vasoconstriction due to upper airway obstruction, and an imbalance between pulmonary vasoconstriction and relaxation (53-57).…”

Section: Pulmonary Vascular Resistance and Capacitance In The Specifi...mentioning

confidence: 99%

“…These specific factors lead to a reduction in capillary recruitment, ensuring a left-downwardshifted RC coupling curve. Notably, low preoperative Cp is an independent predictor for the requirement of postoperative home oxygen therapy, which is introduced in 10%-40% patients of patients with CHD and trisomy 21 (25,27,61). Meanwhile, in trisomy 18, which is the second most common chromosomal aneuploidy, Rp and Cp values are comparable to those in subjects without chromosomal anomalies (28).…”

Section: Pulmonary Vascular Resistance and Capacitance In The Specifi...mentioning

confidence: 99%

Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease

Muneuchi

Ezaki²,

Sugitani³

et al. 2022

Front. Pediatr.

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Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular obstructive disease, and genetic variations of vasoactive mediators. Pulmonary input impedance consists of the pulmonary vascular resistance (Rp) and capacitance (Cp). Rp is calculated as the transpulmonary pressure divided by the pulmonary cardiac output, whereas Cp is calculated as the pulmonary stroke volume divided by the pulmonary arterial pulse pressure. The plots of Rp and Cp demonstrate a unique hyperbolic relationship, namely, the resistor–capacitor coupling curve, which represents the pulmonary vascular condition. The product of Rp and Cp is the exponential pressure decay, which refers to the time constant. Alterations in Cp are more considerable in CHD patients at an early stage of developing pulmonary hypertension or with excessive pulmonary blood flow due to a left-to-right shunt. The importance of Cp has gained attention because recent reports have shown that low Cp potentially reflects poor prognosis in patients with CHD-PH and idiopathic pulmonary hypertension. It is also known that Cp levels decrease in specific populations, such as preterm infants and trisomy 21. Therefore, both Rp and Cp should be individually evaluated in the management of children with CHD-PH who have different disease conditions.

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Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension

Cited by 2 publications

References 21 publications

Pulmonary vascular resistance and compliance in individuals with trisomy 18

Pulmonary vascular resistance and compliance in individuals with trisomy 18

Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease

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