This case confirms the presence of a prominent crista terminalis in a developing fetus, a variant which was exclusively described in the pediatric and adult populations until now. Although a prominent crista terminalis is a benign variant, the correct identification of this structure has important diagnostic implications. This report aims to increase familiarity with the appearance of a prominent crista terminalis on both fetal echocardiography and newborn transthoracic echocardiography in hopes that this will aid in the correct diagnosis of this variant in the future.
Anomalies of the coronary arteries are rare but are an important cause of sudden cardiac arrest in young athletes. Sudden cardiac arrest has been reported in patients with congenital anomalies of the coronary arteries. We present a rare case of sudden cardiac arrest caused by anomalous left main coronary artery originating high from the posterior aspect of left sinus of Valsalva with intramural retroaortic segment, associated with bicuspid aortic valve.
Pseudoaneurysm of the right ventricular outflow tract (RVOT) has been reported as a rare complication of RVOT reconstruction performed using conduit replacement or patch repair. The RVOT needs to be followed up on echocardiography and if aneurysm a large aneurysm forms, it needs to be operated. Usually progression of aneurysm is slow. Rarely, it may present with symptoms secondary to the compression of adjoining mediastinal structures or thrombus formation inside the aneurysm. We report two cases of rapidly progressing massive RVOT aneurysm needing early surgical intervention. Both patients had ventricular septal defect (VSD) closure and right ventricle to pulmonary artery conduit placement. In both patients Contegra pulmonary valved conduit (Medtronic) had been used.
Aorto-left ventricular tunnel is a rare congenital cardiac anomaly, consisting of a short abnormal pathway, usually from a sinus of Valsalva into the left ventricular cavity. It is usually diagnosed with echocardiography. We report a case of a newborn presenting with heart murmur and rapid progression to heart failure and left ventricular enlargement due to an aorto-left ventricular tunnel. Despite successful closure of the tunnel, the patient required a Ross procedure due to progressive aortic disease.
Delineation and documentation of anatomy in the presence of significant mass pathology presents a diagnostic challenge. This often necessitates the implementation of more than one imaging modality in order to perform an adequate assessment. We present a three-year old boy with extensive distortion of mediastinal anatomy secondary to pleural metastases from a Wilms tumour. This limited the ability to accurately assess mediastinal anatomy and cardiac function at baseline. Reassessment following initiation of chemotherapy showed a significant reduction in size of metastases with complete resolution of the mediastinal distortion.
We report a 12-year-old child diagnosed with mycotic aneurysm at the site of coarctation of aorta (CoA) repair. Surgical intervention, though very high risk, is the mainstay of management. In selected cases endovascular stenting is being reported in adults. Here we report the use of an endovascular stent in a child.
We report a 4 year old child referred for routine cardiology evaluation as part of evaluation of murmur with scoliosis. On examination, there was mild duskiness. Echocardiography revealed bilateral superior vena cavae, a small innominate vein, a left superior vena cava to the left atrium and an intact coronary sinus. The findings were confirmed on computed tomography angiography. Percutaneous closure of the left superior vena cava by an Amplatzer vascular plug was done successfully. Although the patient was apparently asymptomatic, the procedure was done to prevent risk of cerebral emboli.
BACKGROUND:Infants with critical left sided obstructive lesions usually present with left ventricle dysfunction and pulmonary arterial hypertension (PAH). Left ventricular dysfunction and pulmonary artery pressures usually normalize after relief of obstruction. In some, PAH persists despite adequate relief of obstruction.PATIENTS AND METHODS:We retrospectively reviewed records of infants (less than 3 months) who underwent intervention for left sided obstruction (n=50) over four years (May 2012-May 2016).RESULT:We report four infants who had persistent of PAH despite relief of left sided obstruction. The causes of PAH were found to be high left ventricular end diastolic pressure(n=1), severe hypoplasia of pulmonary veins (n=1), stenosis of the lower branch of the right pulmonary artery (n=1) and non-regression of pulmonary vascular resistance (n=1).CONCLUSION:That persistence of PAH after relief of obstruction should be investigated and other, less common causes should be sought.
We present a rare case of isolated right subclavian artery arising from a right-sided patent arterial duct in a patient with DiGeorge syndrome, diagnosed on cardiac CT, along with potential complications and management approaches.
Background Congenital Heart Disease (CHD) is the commonest group of congenital malformations and affects 7-8 per 1000 live born newborns. Nevertheless, it is estimated that more than 50% of babies with undiagnosed CHD are not detected by routine neonatal cardiac examination. Aim To find the incidence of CHD in newborns and to determine the accuracy of pulse oximetry for detecting clinically unrecognized critical congenital heart disease (CCHD) in the newborns. Methods Pulse oximetry was performed on clinically normal newborns within 4 hours of first day of life. Inclusion criteria: All newborns who were admitted in postnatal ward & Neonatal Intensive care unit (NICU). Exclusion criteria: babies and neonates with a prenatal diagnosis of duct dependent circulation. If oxygen saturation (SpO2) was below 90%, then echocardiography was performed. Results During the study period, 4926 live born neonates were examined. Nine out of 12 neonates with SpO2<90% had CCHD. Four neonates had tetralogy of Fallot (TOF), two had tricuspid atresia, two had transposition of great arteries (TGA) and one had truncus arteriosus. The incidence of CHD was 33.49 per 1000 live births and CCHD was 1.82 per 1000. A pulse oximetry cut-off value of below 90% for detecting CCHD showed 90% sensitivity, 99.94% specificity, 75% positive predictive value (PPV) and 99.98% negative predictive value (NPV). Conclusion Pulse oximetry is safe, feasible and noninvasive and also used to screen for CCHD. It is the nice method to detect the CHD along with the physical examination of neonates by medical personal.
DiGeorge syndrome is polytopic developmental field defect which is usually associated with 22q11.2 microdeletion. However, this phenotype may be caused by other conditions. We report such a case and briefly review these alternative causes for this particular phenotype.
We present two cases of severe aortic coarctation detected in adulthood and who underwent successful relief by transcatheter stent deployment using a new covered stent, Optimus® stent (AndraTec GmbH Koblenz, Germany). One patient is a 46-year old female with resistant arterial hypertension, in whom coarctation was suspected on a follow-up transthoracic echocardiogram for bicuspid aortic valve disease and subsequently confirmed on magnetic resonance imaging. The second patient is a 68-year old male whose coarctation was diagnosed incidentally at coronary angiography being performed as part of the preoperative work-up for severe aortic stenosis. Suprasternal views to assess aortic arch and flows in the proximal descending aorta should be a standard part of every transthoracic echocardiogram. Treatment of aortic coarctation should be considered irrespective of patient's age.
BACKGROUND:The Reveal LINQ™Insertable Cardiac Monitor (ICM) or Implantable Loop recorder (ILR), is a miniaturized, subcutaneous, single lead, electrocardiographic monitoring device which has been extensively used in the differential diagnosis of unexplained syncope and palpitations in adults.PATIENT DESCRIPTION:We describe an asymptomatic 20-month-old boy, noted to have incidental bradycardia on routine examination and in whom Holter monitoring revealed complete heart block (CHB). Over 1 year, the longest recorded pause lengthened from 1.8 seconds to 3.6 seconds.RESULTS:The Reveal LINQ™ ICM was inserted for long-term monitoring of the CHB. The device will record the electrocardiogram (ECG) continuously for up to 3 years, freezing in its memory any significant arrhythmic events. This will enable the diagnosis of the longest pauses, confirm whether they are lengthening over time and assist with the decision of pacemaker implantation.CONCLUSION:The Reveal LINQ™ ICM is much smaller than the conventional loop recorder and has been shown to be ideal for close monitoring of asymptomatic yet potentially dangerous arrhythmias in young children.
INTRODUCTION:Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage.CASE PRESENTATION:A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation.DISCUSSION:Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied.
