Project description:Twenty patients with various forms of congenital heart disease admitted to Army Hospital Delhi Cantt were studied. After detailed clinical examination and routine investigations they were subjected to two dimensional and subsequently colour Doppler echocardiography. Two patients were operated on the data obtained by echocardiographi. studies only. Seven patients required preoperative catheterisation in addition to echographic examination. The operative procedures confirmed the lesions delineated on colour Doppler echocardiography. Three patients are on echocardiographic review and remaining seven are awaiting surgery to further confirm the diagnosis offered on colour Doppler echo. One pateint of complex lesion died before detailed evaluation could be carried out. Thus colour Doppler echocardiography, though considered highly accurate and non invasive investigation for assessment of congenital heart disease is still in the stage of infancy in paediatric set up and requires a high degree of expertise before it can replace preoperative catheterisation in majority of cases.
Project description:Three-dimensional (3D) whole heart techniques form a cornerstone in cardiovascular magnetic resonance imaging of congenital heart disease (CHD). It offers significant advantages over other CHD imaging modalities and techniques: no ionizing radiation; ability to be run free-breathing; ECG-gated dual-phase imaging for accurate measurements and tissue properties estimation; and higher signal-to-noise ratio and isotropic voxel resolution for multiplanar reformatting assessment. However, there are limitations, such as potentially long acquisition times with image quality degradation. Recent advances in and current applications of 3D whole heart imaging in CHD are detailed, as well as future directions.
Project description:ObjectiveFetal intelligent navigation echocardiography (FINE) is a novel method that automatically generates and displays 9 standard fetal echocardiographic views in normal hearts by applying intelligent navigation technology to spatiotemporal image correlation (STIC) volume data sets. The main objective was to determine the sensitivity and specificity of FINE in the prenatal detection of congenital heart disease (CHD).MethodsA case-control study was conducted in 50 fetuses with a broad spectrum of CHD (cases) and 100 fetuses with normal hearts (controls) in the second and third trimesters. Using 4-dimensional ultrasound with STIC technology, volume data sets were acquired. After all identifying information was removed, the data sets were randomly distributed to a different investigator for analysis using FINE. The sensitivity and specificity for the prenatal detection of CHD, as well as positive and negative likelihood ratios were determined.ResultsThe diagnostic performance of FINE for the prenatal detection of CHD was: sensitivity of 98% (49 of 50), specificity of 93% (93 of 100), positive likelihood ratio of 14, and negative likelihood ratio of 0.02. Among cases with confirmed CHD, the diagnosis with use of FINE completely matched the final diagnosis in 74% (37 of 50); minor discrepancies were seen in 12% (6 of 50), and major discrepancies were seen in 14% (7 of 50).ConclusionsThis is the first time the sensitivity and specificity of the FINE method in fetuses with normal hearts and CHD in the second and third trimesters has been reported. Because FINE identifies a broad spectrum of CHD with 98% sensitivity, this method could be used prenatally to screen for and diagnose CHD.
Project description:IntroductionThe prevalence of defects and effective radiation dose from various myocardial perfusion imaging (MPI) strategies in congenital heart disease (CHD) is unknown.MethodsWe studied 75 subjects with complex CHD (ages 5 to 80 years) referred for MPI between 2002 and 2015. A rest and exercise or pharmacologic stress MPI was performed using 99mTechnetium sestamibi, 82rubidium or 13N-ammonia, and Sodium iodide SPECT (single-photon emission computed tomography), SPECT/CT or Cadmium zinc telluride (CZT) SPECT or PET (positron emission tomography)/CT scanners. Deidentified images were interpreted semi-quantitatively in three batches: stress only MPI, stress/rest MPI, and stress/rest MPI with taking into account a history of ventricular septal defect repair. Effective radiation dose was estimated for stress/rest MPI and predicted for 1-day stress-first (normal stress scans), and for 2-day stress/rest MPI (abnormal stress scans).ResultsThe median age was 18.6 years. The most common type of CHD was transposition of the great arteries (63%). Rest/stress MPI was abnormal in 43% of subjects and 25% of the abnormal scans demonstrated reversible defects. Of the subjects with abnormal MPI, 33% had significant underlying anatomic coronary artery obstruction. Estimated mean effective radiation dose ranged from 2.1 ± 0.6 mSv for 13N-ammonia PET/CT to 12.5 ± 0.9 mSv for SPECT/CT. Predicted effective radiation dose was significantly lower for stress-first MPI and for 2-day stress/rest protocols.ConclusionsDue to the relatively high prevalence of abnormal stress MPI, tailored protocols with a stress-first MPI as well as the use of 2-day protocols and advanced imaging technologies including CZT SPECT, novel image reconstruction software, and PET MPI could substantially reduce radiation dose in complex CHD.
Project description:Fetal dextrocardia is a type of cardiac malposition where the major axis from base to apex points to the right side. This condition is usually associated with a wide spectrum of complex cardiac defects. As a result, dextrocardia is conceptually difficult to understand and diagnose on prenatal ultrasound. The advantage of four-dimensional sonography with spatiotemporal image correlation (STIC) is that this modality can facilitate fetal cardiac examination. A novel method known as fetal intelligent navigation echocardiography (FINE) allows automatic generation of nine standard fetal echocardiography views in normal hearts by applying intelligent navigation technology to STIC volume datasets. In fetuses with congenital heart disease, FINE is also able to demonstrate abnormal cardiac anatomy and relationships when there is normal cardiac axis and position. However, this technology has never been applied to cases of cardiac malposition. We report herein for the first time, a case of fetal dextrocardia and situs solitus with complex congenital heart disease in which the FINE method was invaluable in diagnosing multiple abnormalities and defining complex anatomic relationships. We also review the literature on prenatal sonographic diagnosis of dextrocardia (with an emphasis on situs solitus), as well as tricuspid atresia with its associated cardiac features.
Project description:BackgroundDespite its potential, pediatric three-dimensional (3D) transesophageal echocardiography (TEE) faced technical limitations due to the size and rigidity of early probes designed for adults.AimIn this paper, we present our experience in using the new pediatric 3D TEE probe in complex congenital heart disease (CHD).MethodsRecent advancements in the field of miniaturization have led to the development of a new pediatric 3D TEE probe, specifically designed for young children (weight ≥4.5 kg). This probe features a smaller, flexible design and high-spatial and temporal resolution, enabling detailed views of intracardiac structures and real-time imaging crucial for complex diagnosis, planning interventional and surgical procedures. We used this new probe in pediatric cases (weight between 4.5 and 6 kg) with complex congenital heart disease.ResultsIn double-outlet right ventricle or in Taussig Bing anomaly the new 3D TEE probe enabled detailed assessment of ventricular septal defect and its relations with tricuspid valve apparatus, and provided details of mitral valve leaflets not detectable by the transthoracic approach, even in small children.ConclusionsThese cases highlight the probe's ability to provide detailed anatomical information, enhancing surgical planning and outcomes. The pediatric 3D TEE probe has the potential to be a game changer in defining intracardiac anatomy in complex CHD.
Project description:Congenital heart defects (CHD) are the most common congenital anomaly, and the majority can be diagnosed during prenatal life. Prenatal detection rates remain highly variable, as most CHD occur in low risk pregnancies and therefore depend on the maternal obstetric provider to recognize fetal cardiac abnormality on obstetric screening anatomic ultrasound. Fetuses with abnormal findings on obstetric screening anatomic ultrasound and/or risk factors for cardiac disease should be referred for evaluation with fetal echocardiography. Fetal echocardiography should be performed by specialized sonographers and interpreted by physicians with knowledge of evolving fetal cardiac anatomy and physiology throughout gestation. A fetal echocardiography examination, which can be done from the late first trimester onward, utilizes a standardized and systemic approach to diagnose fetuses with CHD or other forms of primary or secondary cardiac disease. The field of fetal cardiology has advanced past the accurate prenatal diagnosis of simple and complex CHD, as fetal echocardiography enables understanding of dynamic fetal cardiac physiology and consideration of potential fetal/neonatal treatment. The greatest impact of fetal echocardiography remains identification of critical CHD before birth to allow immediate cardiac management after delivery to decrease neonatal morbidity and mortality. Analyzing the severity of abnormal cardiac physiology in various forms of CHD before birth allows the fetal cardiologist to prognosticate effects on the developing fetus, predict risk of postnatal hemodynamic instability, guide delivery planning through multidisciplinary collaboration, and anticipate how the disease will impact the neonate after delivery.
Project description:The lymphatic system plays a central role in some of the most devastating complications associated with congenital heart defects. Diseases like protein-losing enteropathy, plastic bronchitis, postoperative chylothorax, and chylous ascites are now proven to be lymphatic in origin. Novel imaging modalities, most notably, noncontrast magnetic resonance lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography, can now depict lymphatic anatomy and function in all major lymphatic compartments and are essential for modern therapy planning. Based on the new pathophysiologic understanding of lymphatic flow disorders, innovative minimally invasive procedures have been invented during the last few years with promising results. Abnormal lymphatic flow can now be redirected with catheter-based interventions like thoracic duct embolization, selective lymphatic duct embolization, and liver lymphatic embolization. Lymphatic drainage can be improved through surgical or interventional techniques such as thoracic duct decompression or lympho-venous anastomosis.
Project description:The increasing number of survivors of congenital heart disease (CHD) has been paralleled by advancement of imaging modalities used for the ongoing assessment of these patients. There has been a large body of literature describing new approaches to non-invasive assessment of CHD. We will review new applications of well established as well as novel techniques for the management and understanding of CHD.
Project description:Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. PH was determined by cardiac catheterization data or echocardiography reports. In accord with the World Symposia on Pulmonary Hypertension, CHD was further categorized as simple, severe, or complex CHD (including pulmonary atresia-ventricular septal defect and single-ventricle anomalies). There were 4301 patients (55.6% women), 15.7% with severe and 3.9% with complex CHD. The cumulative incidence of PH was 4.4% (95% CI, 3.8-5.0). Our multivariable regression model indicated 4.2-fold mortality increase (95% CI, 3.0-5.9) in the presence of PH, with age, female sex, and severe or complex CHD linked to higher incidence of PH. Only 49% of patients received PH-specific therapy. Five- and 10-year survival rates of patients with PH (n=190) were 72.3% (95% CI, 65.1%-78.4%) and 58.8% (95% CI, 50.1%-66.5%), respectively. Survival rates in those with Eisenmenger syndrome, PH after defect correction, and complex CHD were similar. Low oxygen saturation and high uric acid levels were associated with increased mortality. Conclusions In this sizable Asian adult CHD cohort, the cumulative incidence of PH was aligned with that of Western countries. Mortality proved higher in patients with PH versus without PH. Although complex CHD carried greater risk of PH compared with other adult CHD subsets, survival rate was similar.