Congenital Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins Associated with Hypoplastic Left Heart Syndrome

2001 ◽  
Vol 4 (2) ◽  
pp. 167-174 ◽  
Author(s):  
R. Rabah ◽  
J.M. Poulik

Three full-term infants died in the first month of life with hypoplastic left heart syndrome (HLH) and persistent pulmonary hypertension (PPH). At postmortem examination, they were found to have alveolar capillary dysplasia with misalignment of pulmonary veins (ACD with MPV). The association of HLH syndrome, and ACD with MPV with intestinal malrotation and/or obstruction, is unique. Decreased blood flow in the ascending aorta in fetuses with left outflow tract obstruction might cause vasoconstriction of pulmonary arterioles to maintain cerebral perfusion. Vasoconstriction early during embryo-genesis might lead to decreased growth and development of alveolar capillaries and pulmonary veins. This results in pulmonary hypertension, and the arterial blood is forced to bypass the deficient capillary bed and can drain only via the anomalous bronchial veins.

2007 ◽  
Vol 17 (2) ◽  
pp. 189-195 ◽  
Author(s):  
Amanda J. Shillingford ◽  
Richard F. Ittenbach ◽  
Bradley S. Marino ◽  
Jack Rychik ◽  
Robert R. Clancy ◽  
...  

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criterions for inclusion, with 15 (12%) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Gencheng Zhang ◽  
Glen Van Arsdell ◽  
Helen Holtby ◽  
Sally Cai ◽  
Osman Al Radi ◽  
...  

Aortic atresia (AA) has been identified as a risk factor for mortality after the Norwood procedure. We compared postoperative systemic and regional (cerebral and splanchnic) oxygen transport profiles in neonates with hypoplastic left heart syndrome with or without AA after the Norwood procedure. Systemic oxygen consumption (VO 2 ) was measured using respiratory mass spectrometry postoperatively for 72 hrs in 17 neonates (n=9 in AA group and 8 in non-AA group). Arterial, superior vena cava, and pulmonary venous blood gases and pressures were measured at 2– 4 hr intervals to calculate cardiac output (CO), systemic vascular resistance (SVR), oxygen delivery (DO 2 ) and oxygen extraction ratio (ERO 2 ). Cerebral (ScO 2 ) and splanchnic (SsO 2 ) oxygen saturations were measured using near infrared spectroscopy. The doses of dopamine, vasopressin (up to 0.0008 unit/kg/min), phenoxybenzamine and milrinone were noted. When compared to non-AA group, AA group had lower CO (p=0.03), higher systolic arterial blood pressure (p=0.01) and SVR (p=0.002), lower DO 2 (p=0.07), VO 2 (p=0.003) and ScO 2 during the first 30 to 40 hrs. There was no significant difference in SsO 2 (p=0.12, n=7 in AA group and 3 in non-AA group). Despite a similar ERO 2 (p=0.5), AA group had higher lactate over 72 hrs (p=0.01). AA group received higher doses of vasopressin (p=0.005) and milrinone (p<0.0001), lower doses of dopamine (p=0.07), with similar use of phenoxybenzamine. Furthermore, VO 2 negatively correlated with vasopressin (p=0.05), positively correlated with milrinone (p=0.03) and dopamine (p<0.0001). When including the drugs, the inferior status in all the oxygen transport variables remained, and additionally adversely correlated with vasopressin (p from <0.0001 to 0.06), except for ScO 2 that positively correlated with dopamine, but not with other drugs. Aortic atresia is associated with an inferior status of systemic, cerebral and splanchnic oxygen transport in neonates after the Norwood procedure. Aggressive use of vasopressin worsens systemic and splanchnic oxygen transport status. Postoperative management strategies should be directed to improve both systemic and regional oxygen transport in this special group of patients.


2020 ◽  
Vol 32 (3) ◽  
pp. 531-538
Author(s):  
Elizabeth H. Stephens ◽  
Dipankar Gupta ◽  
Mark Bleiweis ◽  
Carl L. Backer ◽  
Robert H. Anderson ◽  
...  

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