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Biomedical subjects

James C Huhta

Publications and source records attributed to James C Huhta.

13 recordsLinked to original sources

Update in fetal cardiac intervention.

The severity of fetal heart disease progresses during gestation and may lead to significant in utero or postnatal morbidity and mortality. Fetal echocardiography allows us to detect heart disease early enough in pregnancy to perform fetal cardiac intervention that may change the natural history of some diseases. The principal aims are the prevention of hydrops due to congenital heart disease, recruitment of hypoplastic ventricles, remodeling of the fetal pulmonary vascular bed, or creation of a two-ventricular circulation after birth without risk to the mother. The initial fetal interventions for valvuloplasty and atrial septostomy showed a high mortality and did not achieve significant change in the final outcome of patients. Nevertheless, some technique modifications have improved the outcome of these patients, making fetal cardiac intervention a promising treatment for congenital heart disease. Conversely, different assessment tools for selection of the correct patient have been studied in some series to improve the success of these procedures and thus the survival odds. We believe that percutaneous procedures with local anesthesia are preferable.

Journal Article↗

A cardiovascular profile score in the surveillance of fetal hydrops.

OBJECTIVE: To assess the value of a cardiovascular profile score in the surveillance of fetal hydrops. METHODS: In a retrospective study, 102 hydropic fetuses were examined between 15 and 37 completed weeks of gestation with ultrasonographic assessment of hydrops, heart size, and cardiac function, and arterial umbilical and venous Doppler sonography of the ductus venosus (DV) and the umbilical vein (UV). A cardiovascular profile score (CVPS) was constructed by attributing 2 points for normal and taking away 1 or 2 points for abnormal findings in each category. The score of the final examination prior to treatment, delivery, or fetal demise was compared to the fetal outcome in these 102 fetuses after exclusion of terminated pregnancies. The scores of the first and last examinations were compared in 40 fetuses and the relationship between these scores and the evolution of fetal hydrops and fetal outcome was assessed. RESULTS: Twenty-one pregnancies were terminated (21%). Fifty-four of the remaining 81 hydropic fetuses survived (67%) and perinatal death (PNM) occurred in 27 fetuses (33%). The median CVPS was 6.0 (IQR 4.75-8.00) for all fetuses, with a median of 6.0 (IQR 5.00-6.00) in fetuses who died in the perinatal period compared to a median of 7.0 (IQR 4.00-8.00) in those who survived (p < 0.035). All fetuses in this study had a 'severe' form of hydrops with skin edema. The best predictor for adverse outcome was the venous Doppler sonography of UV and DV, in particular umbilical venous pulsations. Among fetuses included in the longitudinal arm of the study, the survival rate was 40% and the PNM was 60%, after exclusion of terminated pregnancies. CVPS increased by a median of 1 (IQR 0.00-2.00) point in the last exam for those fetuses that lived, whereas among those fetuses that died, the CVPS decreased by a median 1.5 (IQR 0.25-2.75) points (p < 0.001). CONCLUSIONS: The fetal cardiovascular profile score can be used in the surveillance of hydropic fetuses for prediction of the presence of congestive heart failure and as an aid for predicting fetal outcome.

Cardiomegaly↗

Fetal congestive heart failure.

Fetal echocardiography is used in the diagnosis of many forms of congenital heart disease, and in the assessment of the prognosis of cardiac lesions based on their anatomy and presentation in utero. However, the presence of signs of fetal heart failure such as hydrops or valvular regurgitation makes the assessment of prognosis more difficult. A tool for this assessment is the 'cardiovascular profile score', which combines ultrasonic markers of fetal cardiovascular unwellness based on univariate parameters that have been correlated with perinatal mortality. This profile could become the 'heart failure score' and could potentially be used in much the same way as and in combination with the biophysical profile score. This article will present a straightforward method for rapid evaluation of a fetus that may have congestive heart failure.

Blood Flow Velocity↗

The hemodynamic effects of neonatal patent ductus arteriosus shunting on superior mesenteric artery blood flow.

OBJECTIVE: To determine if the ratio of the pulsatility index (PI) of the left pulmonary artery to the PI of the descending aorta, the Rp/Rs index, correlates with the degree of ductal steal from the intestine in neonates with a patent ductus arteriosus (PDA). STUDY DESIGN: Echocardiograms and Doppler studies of the superior mesenteric artery (SMA) were performed in 41 neonates less than 35 weeks gestational age with a hemodynamically significant PDA (hsPDA). RESULTS: There was a significant negative correlation between the Rp/Rs index and the SMA PI after controlling for ductal size (r=-0.476, p<0.008). CONCLUSIONS: The Rp/Rs index can be used as an indicator of ductal steal on intestinal blood flow. The Rp/Rs index may be a useful adjunct to existing and new techniques for improving early assessment and treatment of hsPDA, and for evaluating the effects of hsPDA on systemic organs.

Aorta, Thoracic↗

The ontogeny of biochemical markers of cardiac dysfunction.

PURPOSE OF REVIEW: Biochemical markers are available to detect cardiac involvement in many pediatric disease states and should be considered. RECENT FINDINGS: Analyses of three markers are readily available in clinical laboratories for improved diagnosis. SUMMARY: Increased workload of the heart has been associated with the release of biochemical markers (natriuretic peptides and cardiac enzymes) that indicate that a new genetic program has been activated and maladaptation is occurring in the atria, ventricles, or both. This review summarizes those that have been identified in fetal and pediatric practice. The expression of such markers is traced from early embryonic development to fetal life, to the neonate, to childhood, and then to adult life.

Adolescent↗

The use of pulse oximetry to detect congenital heart disease.

OBJECTIVE: To evaluate whether pulse oximetry can be used as a screening test to detect congenital heart disease (CHD) in otherwise well newborns before nursery discharge. METHODS: Births at Lakeland Regional Medical Center (LRMC) who met inclusion criteria, had a single pulse oximeter reading before discharge. A normal reading was considered to be >94%. Abnormal readings were repeated on a different extremity and if still abnormal, an echocardiography was performed. The number of children with CHD and the number of echocardiograms were compared with the previous year at LRMC. RESULTS: There were 2114 infants enrolled, 88 echocardiograms (3.8%) were performed, and 43 were abnormal (yield = 48.9%), of which 12 required management (13.6%). The control group consisted of 2851 births, producing 108 echocardiograms (3.8%), 42 of which were abnormal (38.9%), and 13 required management (12.0%). No comparisons were significant. During both years, 3 children with cyanotic CHD were born. Routine pulse oximetry failed to detect a child with total anomalous pulmonary venous return. Not a single normal echocardiogram was generated by the study. CONCLUSION: Routine pulse oximetry was nearly 100% specific for detecting cyanotic CHD; as a result there was no increase in the number of echocardiograms. Routine pulse oximetry did detect a child who might not have been detected otherwise but failed to detect one that should have been detected.

Cyanosis↗

Clinicopathologic conference: Barth Syndrome.

A case of Barth Syndrome is presented and discussed by both clinician and pathologist, in this traditional clinico-pathologic conference. The current understanding of etiology is included, including elevation of 3-methylglutaconic acid (3MGC).

Abnormalities, Multiple↗

Homocysteine, folate, and congenital heart defects.

Congenital heart disease will be the next frontier for prevention by periconceptional management of homocysteine and its metabolites by folate supplementation. Evidence for the connection between maternal and fetal folate metabolism and congenital malformations of the cardiovascular system is reviewed including possible applications to the large population of patients at risk for a child with congenital heart disease.

Animals↗