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

Abstract Objective: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. Methods: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. Results: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of −0.70 (−1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). Conclusions: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

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Pulmonary arterial resistance and compliance in preterm infants

International Journal of Cardiology ◽

10.1016/j.ijcard.2017.06.056 ◽

2017 ◽

Vol 244 ◽

pp. 265-270 ◽

Cited By ~ 4

Author(s):

Seigo Okada ◽

Jun Muneuchi ◽

Yusaku Nagatomo ◽

Mamie Watanabe ◽

Chiaki Iida ◽

...

Keyword(s):

Preterm Infants ◽

Arterial Resistance ◽

Pulmonary Arterial

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Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2016.03.080 ◽

2016 ◽

Vol 152 (2) ◽

pp. 507-513 ◽

Cited By ~ 5

Author(s):

Jun Muneuchi ◽

Yusaku Nagatomo ◽

Mamie Watanabe ◽

Kunihiko Joo ◽

Tatsushi Onzuka ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Arterial Resistance ◽

Pulmonary Arterial

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Distribution of lung vascular resistance after chronic systemic-to-pulmonary shunts

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.1985.249.6.h1106 ◽

1985 ◽

Vol 249 (6) ◽

pp. H1106-H1113 ◽

Cited By ~ 1

Author(s):

R. P. Michel ◽

T. S. Hakim ◽

R. E. Hanson ◽

A. R. Dobell ◽

F. Keith ◽

...

Keyword(s):

Arterial Pressure ◽

Vascular Resistance ◽

Pulmonary Circulation ◽

Pulmonary Arterial Pressure ◽

Lower Lobe ◽

Venous Occlusion ◽

Morphometric Measurements ◽

Arterial Resistance ◽

Pulmonary Arterial ◽

The Right

Congenital cardiac shunts produce pathological lesions on the arterial side of the lung vasculature. We examined the effects of chronic shunts (14.2 +/- 1.2 mo) in 10 young dogs, between the left subclavian and the left lower lobe (LLL) artery, on pulmonary vascular pressure and flow (P-Q) relationships, segmental resistance with arterial and venous occlusion (AVO), and sensitivity to drugs. At final thoracotomy, mean LLL pulmonary arterial pressure (Ppa) was 23.2 +/- 4.3 mmHg compared with 11.9 +/- 0.9 in the right lung (P less than 0.05); two animals had LLL Ppa of 41 and 48 mmHg. The LLL artery and vein were cannulated, and pressure-flow (P-Q) and AVO measurements were made and compared with previous control LLL (n = 11) and contralateral right lower lobe (RLL, n = 5). Responses to serotonin, histamine, and vasodilators (diltiazem and isoproterenol) were evaluated. Comparisons of morphometric measurements were made between LLL and RLL. We found a significant increase in arterial resistance as measured with AVO and a hypersensitivity to serotonin in the shunt LLL, without changes in total pulmonary vascular resistance or P-Q measurements; vasodilators had a small effect only in the hypertensive lobes. Our data suggest that chronic shunts to the pulmonary circulation increase arterial resistance and sensitivity to serotonin, even in the absence of discernible morphometric changes, and that vasoconstriction may be an important precursor to the development of morphological lesions.

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Pulmonary Arterial Resistance: Noninvasive Measurement with Indexes of Pulmonary Flow Estimated at Velocity-encoded MR Imaging—Preliminary Experience

Radiology ◽

10.1148/radiology.212.3.r99au21896 ◽

1999 ◽

Vol 212 (3) ◽

pp. 896-902 ◽

Cited By ~ 86

Author(s):

Elie Mousseaux ◽

Jean Pierre Tasu ◽

Odile Jolivet ◽

Gérard Simonneau ◽

Jacques Bittoun ◽

...

Keyword(s):

Mr Imaging ◽

Noninvasive Measurement ◽

Arterial Resistance ◽

Pulmonary Flow ◽

Pulmonary Arterial

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Advanced therapies in pulmonary arterial hypertension and congenital heart disease in people with Down syndrome

Journal of Congenital Cardiology ◽

10.1186/s40949-020-00051-x ◽

2020 ◽

Vol 4 (S1) ◽

Author(s):

Zoya Rashid ◽

Robert Tulloh

Keyword(s):

Congenital Heart Disease ◽

Down Syndrome ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Response To Therapy ◽

Therapeutic Decisions ◽

Advanced Therapies ◽

Pulmonary Arterial

Abstract Background Management of patients with pulmonary arterial hypertension associated with congenital heart disease and trisomy 21 is complex due to uncertainty over the best first-line agent to use for treatment, and the outcome measures for assessing response to therapy. There are also ethical issues around therapeutic decisions which may require ‘best interests’ meetings of carers and health care professionals. Case presentation We present a case of a 35-year-old man with Down syndrome, with un-operated atrioventricular septal defect, right ventricular hypertrophy and moderate left atrioventricular valve regurgitation. In the clinic he was visibly cyanosed with peripheral oxygen saturations of 78% at rest. He had very limited walk distance and was in WHO class III. Conclusions We discuss the decisions made around the best therapy for his Eisenmenger syndrome taking into account patient and family views on therapy and considering the psychosocial burden of treatment.

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Is Down Syndrome Related to Pulmonary Arterial Hypertension? A Comparative Study

ACTA MEDICA IRANICA ◽

10.18502/acta.v59i5.6662 ◽

2021 ◽

Author(s):

Ghazaleh Doostparast Torshizi ◽

Mahboobe Gholami ◽

Behzad Alizadeh

Keyword(s):

Down Syndrome ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Comparison Group ◽

Pulmonary Arterial Pressure ◽

Cross Sectional Study ◽

Pulmonary Vascular Disease ◽

Cross Sectional ◽

Pulmonary Arterial ◽

And Gender

Down syndrome (DS) is a genetic impairment associated with comorbidities such as Congenital Heart Disease (CHD). Pulmonary Arterial Hypertension (PAH) is a complication of CHD in most patients. Due to insufficient documents about the prevalence of PAH in DS with CHDs compared to non-DS (NDS)+CHD patients, this study aimed to compare the prevalence of PAH between DS-CHD and NDS-CHD patients. This is a cross-sectional study conducted on DS-CHD patients referred to the Pediatric and Congenital Cardiology Division at Imam Reza training hospital in Mashhad, Iran, between April 2015 and February 2016. The comparison group included NDS-CHD children matched in terms of age and gender. A comprehensive Echocardiography was run for all patients to determine the types of CHD and pulmonary arterial pressure. Seventy-seven patients were enrolled in the study (47 in the DS-CHD group and 30 in the NDS-CHD group). 48.9% of the DS-CHD patients and 23.3% of the NDS-CHD group developed PAH, which revealed a significantly higher rate of PH among DS-CHD patients (P=0.025). Our findings denote a higher prevalence of PAH among DS-CHD patients compared to NDS-CHD patients. Such an observation is a meaningful warning for DS patients to take early necessary medical or corrective therapies for CHD in order to prevent complications and irreversible pulmonary vascular disease.

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Therapeutic hypercapnia prevents chronic hypoxia-induced pulmonary hypertension in the newborn rat

AJP Lung Cellular and Molecular Physiology ◽

10.1152/ajplung.00480.2005 ◽

2006 ◽

Vol 291 (5) ◽

pp. L912-L922 ◽

Cited By ~ 65

Author(s):

Crystal Kantores ◽

Patrick J. McNamara ◽

Lilian Teixeira ◽

Doreen Engelberts ◽

Prashanth Murthy ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Vascular Remodeling ◽

Chronic Hypoxia ◽

Antioxidant Properties ◽

Ventricular Performance ◽

Pulmonary Vascular Remodeling ◽

Beneficial Effects ◽

Arterial Resistance ◽

Pulmonary Arterial

Induction of hypercapnia by breathing high concentrations of carbon dioxide (CO2) may have beneficial effects on the pulmonary circulation. We tested the hypothesis that exposure to CO2 would protect against chronic pulmonary hypertension in newborn rats. Atmospheric CO2 was maintained at <0.5% (normocapnia), 5.5%, or 10% during exposure from birth for 14 days to normoxia (21% O2) or moderate hypoxia (13% O2). Pulmonary vascular and hemodynamic abnormalities in animals exposed to chronic hypoxia included increased pulmonary arterial resistance, right ventricular hypertrophy and dysfunction, medial thickening of pulmonary resistance arteries, and distal arterial muscularization. Exposure to 10% CO2 (but not to 5.5% CO2) significantly attenuated pulmonary vascular remodeling and increased pulmonary arterial resistance in hypoxia-exposed animals ( P < 0.05), whereas both concentrations of CO2 normalized right ventricular performance. Exposure to 10% CO2 attenuated increased oxidant stress induced by hypoxia, as quantified by 8-isoprostane content in the lung, and prevented upregulation of endothelin-1, a critical mediator of pulmonary vascular remodeling. We conclude that hypercapnic acidosis has beneficial effects on pulmonary hypertension and vascular remodeling induced by chronic hypoxia, which we speculate derives from antioxidant properties of CO2 on the lung and consequent modulating effects on the endothelin pathway.

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Pulmonary hypertension and myocardial function in infants and children with Down syndrome

Archives of Disease in Childhood ◽

10.1136/archdischild-2019-318178 ◽

2020 ◽

Vol 105 (11) ◽

pp. 1031-1034

Author(s):

Aisling Mary Smith ◽

Philip T Levy ◽

Orla Franklin ◽

Eleanor Molloy ◽

Afif EL-Khuffash

Keyword(s):

Pulmonary Hypertension ◽

Down Syndrome ◽

Neonatal Period ◽

Myocardial Performance ◽

Early Neonatal Period ◽

At Risk Children ◽

Increased Risk ◽

Infancy And Childhood ◽

Pulmonary Arterial

Down Syndrome (DS) is the most common chromosomal abnormality of live born babies. Individuals with DS are at increased risk of cardiopulmonary morbidities in the early neonatal period, infancy and childhood that manifest with elevated pulmonary arterial pressures and altered myocardial performance. Pulmonary hypertension (PH) during the early neonatal period remains under-recognised in this population. PH may occur with or without a congenital heart defect in children with DS and is more common than in the general population. Early detection and continued screening of PH throughout infancy and childhood for these at-risk children is crucial for prompt intervention and potential prevention of long-term sequelae on cardiac function. This review summarises the main physiological concepts behind the mechanisms of PH in children with DS and provides a summary of the current available literature on PH and its impact on myocardial performance.

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