scholarly journals Non-invasive assessment of liver fibrosis by magnetic resonance elastography in patients with congenital heart disease undergoing the Fontan procedure and intracardiac repair

2016 ◽  
Vol 68 (3) ◽  
pp. 202-208 ◽  
Author(s):  
Masaya Sugimoto ◽  
Hideharu Oka ◽  
Aya Kajihama ◽  
Kouichi Nakau ◽  
Seiko Kuwata ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Magnetic Resonance ◽  
Liver Fibrosis ◽  
Congenital Heart ◽  
Fontan Procedure ◽  
Magnetic Resonance Elastography ◽  
Non Invasive ◽  
Intracardiac Repair

Abstract 12308: Assessment of Liver Fibrosis Using Magnetic Resonance Elastography in Children Who Undergo the Fontan Procedure and Intracardiac Repair

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Masaya Sugimoto ◽  
Hideharu Oka ◽  
Aya Kajihama ◽  
Kouichi Nakau ◽  
Hiroshi Azuma
Keyword(s):  
Magnetic Resonance ◽  
Liver Fibrosis ◽  
Fontan Procedure ◽  
Venous Pressure ◽  
Liver Stiffness ◽  
Magnetic Resonance Elastography ◽  
Cardiac Biomarkers ◽  
Control Group ◽  
Intracardiac Repair ◽  
Significant Difference

Background: The incidence of late complications related to the liver such as fibrosis/cirrhosis is increasing in patients who have undergone the Fontan procedure and may contribute to morbidity and mortality. Recently, magnetic resonance elastography (MRE), a novel evaluation technique of liver fibrosis, has been attracting attention. However, few reports have described the use of MRE for evaluating liver fibrosis in children with congenital heart disease (CHD). Methods: Thirty-two children were examined and divided into 4 groups: 12 children with CHD who underwent intracardiac repair (ICR; median age, 13.0 years); 10 with CHD who underwent the Fontan procedure (Fontan; 15.3 years); 8 who were included in the control group (control; 15.8 years); and 2 children with cirrhosis (cirrhosis; 16.3 years). The liver stiffness (LS) was estimated by MRE. LS was measured 3 times consecutively, and the mean value was considered for further analysis. Central venous pressure (CVP) and cardiac index (C.I.) were determined by cardiac catheterization. The levels of cardiac biomarkers (NTproBNP and PIIIP) were determined at the same time. Results: Among the 4 groups, no significant differences were observed in age, C.I., and NTproBNP levels. The PIIIP levels in the cirrhosis group were significantly higher than those in the control, but no significant difference in PIIIP levels was found among the other groups (p < 0.01). LS in the Fontan and cirrhosis group was significantly higher than that in the control group (5.6, 15.3 vs. 2.4 kPa, respectively; Fig. 1). Furthermore, there was a strong correlation between LS and CVP (r = 0.802; Fig. 2). Conclusions: This study showed that LS is a direct function of CVP, which should be considered when assessing the degree of liver fibrosis in children with CHD. In particular, in the case of children who undergo the Fontan procedure, the highly sensitive MRE can be used to evaluate liver fibrosis and help detect LS earlier than cardiac biomarkers do.

scholarly journals Down's Syndrome with Congenital Heart Disease: Analysis of Cases Over Two Years in a Non-Invasive Laboratory of a Tertiary Hospital

1970 ◽  
Vol 2 (2) ◽  
pp. 184-187
Author(s):  
NN Fatema
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Doppler Echocardiography ◽  
Congenital Heart ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Tertiary Hospital ◽  
Study Group ◽  
Non Invasive ◽  
Study Results

Background: Trisomy 21 or Down's Syndrome is the most frequent chromosomal aberration affecting live birth infants with an incidence of 1 in 660 live births. This syndrome is often associated with congenital cardiac lesions, Incidence of which is 40-60 percent. This study was conducted to see the frequency of Down’s syndrome cases and pattern of heart diseases they have in one of the busy non-invasive pediatric cardiac laboratory of the country. Methods: It was a retrospective study conducted in the non-invasive pediatric cardiac laboratory and pediatric cardiac outpatient clinic of a tertiary hospital over a period of two years (November 2007 to October 2009). The entire patients who had Down's Syndrome and had Doppler echocardiography were included in the study. Results: Out of total six thousand and fifty echocardiography, Down's Syndrome case was 205 (3.38%). Out of 205 cases, 185 cases were followed up in pediatric cardiac out patient clinic. Twenty cases had not reported in the out patient clinic. Seventeen of those patients had normal cardiac anatomy in Doppler echocardiography. Male were 43.90% and female were 56.09% amongst study group. Most of the patients are young infant (47.32%). Only 2.44% are in more than 10 years age group. Murmur was audible in 86.49% cases in study group and developmental delay was present in 100% of the cases. Doppler Echocardiography was found as most sensitive and specific investigation for detecting congenital heart disease. A-V canal defect was the commonest association (15.60%). Congenital heart disease was not detected in 8.29% cases. Surgical treatment was advised in 52.19% cases, Device closure was advised in 16.59% cases, medical management was advised in 21.46% cases. Conclusion: Down's syndrome is a very common chromosomal anomaly in our country. Incidence of this syndrome is increasing as number of working women, late marriage and elderly mother increasing. So, multidisciplinary approach for managing this disease should be adopted immediately. Keywords: Down's syndrome; Congenital heart disease. DOI: 10.3329/cardio.v2i2.6637Cardiovasc. j. 2010; 2(2) : 184-187

Magnetic Resonance Imaging in Children with Congenital Heart Disease

1986 ◽  
pp. 218-224
Author(s):  
Robert A. Boxer ◽  
Michael A. LaCorte ◽  
Mitchell Goldman ◽  
Sharanjeet Singh ◽  
C. Winterfeldt ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Magnetic Resonance ◽  
Congenital Heart ◽  
Resonance Imaging

scholarly journals An unusual cause of edema in a child with congenital heart disease post Fontan procedure

2019 ◽  
Vol 7 (11) ◽  
pp. 4396
Author(s):  
Minal Wade ◽  
Shweta Shettiwar ◽  
Ankita Shah
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Protein Losing Enteropathy ◽  
Common Causes ◽  
Disease Protein ◽  
The Common ◽  
Fontan Palliation

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

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