Circulation In Congenital Heart Disease Research Articles

ACE2 gene polymorphisms are associated with elevated pulmonary artery pressure in congenital heart diseases

BackgroundsAngiotensin converting enzyme 2 (ACE2) polymorphisms are related to the occurrence and prognosis of cardiovascular disease. However, whether ACE2 polymorphisms also affect pulmonary circulation in congenital heart disease (CHD) remains unclear. Thus, we investigated the relationship between ACE2 single nucleotide polymorphism (SNPs) and pulmonary circulation in CHD patients of Chinese Han ethnicity. MethodsEnrolled CHD patients (n = 367) underwent gene sequencing of ACE2 SNPs rs2074192, rs2285666, and rs2106809. Patients with pulmonary hypertension were further examined for detailed hemodynamics. ResultsFemale heterozygous patients had worse pulmonary circulation hemodynamic parameters compared to those of homozygotes. Female CHD patients with the CCA (OR = 0.53, 95% CI: 0.32–0.88) or CCG (OR = 0.59, 95% CI: 0.35–0.99) haplotype had a lower risk of elevated pulmonary artery pressure. ConclusionIn female CHD patients, ACE2 SNPs are related to pulmonary circulation hemodynamics. Female CHD patients with the CCA and CCG haplotype had a lower risk of pulmonary hypertension.

Temporal correlation‐based dynamic contrast‐enhanced MR imaging improves assessment of complex pulmonary circulation in congenital heart disease

A temporal correlation (TC) mapping method is proposed to help bolus chasing during dynamic contrast-enhanced (DCE) MRI of complex pulmonary circulation (CPC) in patients with congenital heart disease. DCE-MRI was performed on five healthy male subjects (23-24 years old) and 25 patients (nine males and 16 females, 0.25-44 years old), and TC maps were generated by performing pixel-based computation of cross-correlations to the pulmonary artery with a series of time shifts in all subjects. Qualitative and quantitative evaluations were performed in comparison with original DCE images. TC maps exhibited a better signal-to-noise ratio (SNR) by factors of 4.3 and 1.3 in the lung parenchyma, pulmonary veins, and superior artery/vein; a better intraparenchymal contrast-to-noise ratio (CNR) by factors of 1.5-5.4; and a significantly higher conspicuity in all regions except the pulmonary arteries when graded with a five-point score. TC maps evaluated by two experienced clinicians significantly added relevant information (P<0.001), and in some cases affected the final diagnosis. We conclude that TC maps facilitate bolus chasing for DCE-MRI by reducing recirculation effects and interframe fluctuations, and hence complements morphological imaging of CPC in patients with complex congenital heart disease.

Effect of Embolization of Major Aortopulmonary Collateral Arteries on Cardiopulmonary Circulation in Congenital Heart Disease

Major aortopulmonary collateral arteries (MAPCAs) may affect pulmonary hemodynamics significantly in patients with cyanotic congenital heart diseases. Occluding such collateral arteries is expected not only to diminish systemic-to-pulmonary shunt but also to increase pulmonary perfusion via the central pulmonary artery. The authors performed coil embolization of MAPCAs communicating with the central pulmonary artery or its branches in 3 patients aged from three to fifteen years (2: tetralogy of Fallot, 1: pulmonary atresia with ventricular septal defect). On chest roentgenogram, the cardiac size diminished after embolization in 2 subjects owing probably to decreased systemic-topulmonary blood flow. The pulmonary perfusion scintigram with Tc 99m macroaggregated albumin was performed before and after embolization. Before embolization, multiple segmental or lobar perfusion defects or hypoperfusion areas were observed in all cases. After embolization, however, the scintigrams unexpectedly did not show any improvement. These results suggest that the occlusion of MAPCAs is effective in decreasing systemic-to-pulmonary blood flow but not effective in increasing lung perfusion. The authors speculate that obliterative and irreversible pulmonary vascular damages might have occurred in all cases.

Book Review: The Pulmonary and Bronchial Circulation in Congenital Heart Disease

Book Review: The Pulmonary and Bronchial Circulation in Congenital Heart Disease

The structure and composition of the pulmonary circulation in congenital heart disease.

The structure and composition of the pulmonary circulation in congenital heart disease.

The effect of carbon dioxide on the pulmonary circulation in congenital heart disease.

The effect of carbon dioxide on the pulmonary circulation in congenital heart disease.

The key to the circulation in congenital heart disease

The key to the circulation in congenital heart disease

Blood volume in congenital heart disease

The abnormal circulation in congenital heart disease is in many respects similar to the abnormal circulation in a peripheral arteriovenous fistula, although the vascular paths of the shunted blood are shorter. This communication is continuous in an extracardiac fistula, as the patent ductus arteriosus, and intermittent in intracardiac shunts, the septal defects. The abnormal communication initiates an accessory circulation which, in relation to dynamics and volume, is independent of the systemic or pulmonary circulation upon which it is parasitic. The accessory or “third” circulation in several simple congenital cardiac defects is illustrated. The relation of the abnormal circulation to the circulating blood volume is considered. The volume of the circulation was determined by the blue dye method in three categories of congenital heart disease, (1) intracardiac acyanotic shunts, (2) patent ductus arteriosus, and (3) cyanotic congenital heart disease. The values obtained were compared with the blood volume of normal children. The blood volume in patients with a patent ductus arteriosus was found to be increased, and the volume diminished following surgical ligation of the open ductus. The blood volume in acyanotic intracardiac arteriovenous shunts was elevated by a smaller amount. The volume in the cyanotic type of congenital heart disease was greatly increased, due to an increase of red blood cells. The plasma was usually below the expected amount. It was concluded that the abnormal circulation in the three categories ofcongenital heart disease requires an elevation of circulating blood volume. It could not be determined whether this increase is a direct measure of the abnormal shunt. The abnormal circulation in congenital heart disease is in many respects similar to the abnormal circulation in a peripheral arteriovenous fistula, although the vascular paths of the shunted blood are shorter. This communication is continuous in an extracardiac fistula, as the patent ductus arteriosus, and intermittent in intracardiac shunts, the septal defects. The abnormal communication initiates an accessory circulation which, in relation to dynamics and volume, is independent of the systemic or pulmonary circulation upon which it is parasitic. The accessory or “third” circulation in several simple congenital cardiac defects is illustrated. The relation of the abnormal circulation to the circulating blood volume is considered. The volume of the circulation was determined by the blue dye method in three categories of congenital heart disease, (1) intracardiac acyanotic shunts, (2) patent ductus arteriosus, and (3) cyanotic congenital heart disease. The values obtained were compared with the blood volume of normal children. The blood volume in patients with a patent ductus arteriosus was found to be increased, and the volume diminished following surgical ligation of the open ductus. The blood volume in acyanotic intracardiac arteriovenous shunts was elevated by a smaller amount. The volume in the cyanotic type of congenital heart disease was greatly increased, due to an increase of red blood cells. The plasma was usually below the expected amount. It was concluded that the abnormal circulation in the three categories ofcongenital heart disease requires an elevation of circulating blood volume. It could not be determined whether this increase is a direct measure of the abnormal shunt.