Intravascular Ultrasonography of an Adolescent Boy with a Coronary Artery Aneurysm Due to Kawasaki Disease

A 14-year-old boy developed coronary aneurysms during the course of Kawasaki disease. The vessel wall morphology of the coronary arteries was assessed by intravascular ultrasound imaging (IVUS). There was intimal thickening proximal to, within, and distal to a large coronary aneurysm in the left anterior descending coronary artery only 18 months after the acute onset of Kawasaki disease. Intimal thickening without calcification is a new IVUS finding within a persistent coronary aneurysm. It may indicate that the healing process, via cell proliferation is continuing, with the risk of causing a stenosis.     

Myocardial Infarction in a 14-Year-Old Girl, Ten Years After Surgical Correction of Congenital Coronary Artery Fistula

Acute myocardial infarction developed in a 14-year-old girl, ten years after surgical repair of a coronary artery fistula. Angiography revealed fresh thrombus in the left anterior descending branch of the left coronary artery. The thrombus probably developed in the residual cul-de-sac of the occluded fistula. A procedure to abolish the cul-de-sac was then performed.     

Cardiac Ventricular Aneurysm in a Child

Cardiac ventricular aneurysms are anomalous developments that are multifactorial in origin. Few pediatric cases have been cited. The majority of the cited cases have had negative consequences. We report an asymptomatic boy with an aneurysm incidentally found, and his identical twin brother with no evidence of aneurysm.     

Aberrant Left Coronary Artery Arising from the Right Sinus of Valsalva with a Right Coronary Arteriovenous Malformation

An 11-year-old boy presented with myocardial ischemia and was found to have an aberrant left main coronary artery from the right sinus of Valsalva coursing between the aorta and pulmonary artery, as well as a small arteriovenous malformation from a right atrial branch of the right coronary artery to the right atrium. Distinctive echocardiographic findings were supported by angiographic and magnetic resonance imaging studies. Treadmill and scintigraphic stress testing were normal. Corrective surgery was accomplished by a modified technique to unroof the intramural proximal course of the left coronary artery, without postoperative complications. The anatomy and pathophysiology of this rare coronary lesion are reviewed.     

Early Presentation of a Congenital Coronary Artery Fistula in a Neonate

A neonate developed severe congestive heart failure secondary to a congenital coronary artery fistula requiring emergent surgery. Intraoperative transesophageal echocardiogaphy helped guide successful emergent closure of the fistula without complications.     

Origin of the Main Pulmonary Artery from the Left Coronary Artery in Complex Pulmonary Atresia

We describe a 22-year-old woman with a history of unrepaired pulmonary atresia with ventricular septal defect. This woman was interesting in that her main pulmonary artery and right pulmonary artery arose from the left main coronary artery. She developed significant pulmonary hypertension in addition to isolation of the left pulmonary artery following ductal closure, subsequent to which the majority of her pulmonary blood flow was coronary dependent.     

Abnormal Coronary Flow Reserve in a 13-Year-Old Girl with an Absent Left Circumflex Coronary Artery

We measured the coronary flow reserve in a 13-year-old girl with the rare anomaly of an absent left circumflex coronary artery. Although the coronary flow volume of the right coronary artery was at the same level as that of the left anterior descending coronary artery, the coronary flow reserve of the patient's right coronary artery was depressed without stenotic lesion, and it was less than the level of −2 standard deviation (SD) of the right coronary artery preponderance cases.     

Anomalous Origin of the Left Coronary Artery in Tetralogy of Fallot Associated with Abnormal Mitral Valve Pathology

The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of Fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.     

Double-Outlet Right Ventricle (Tetralogy of Fallot Type) Associated with Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery: Report of Successful Total Repair in a 2-Month-Old Infant

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.     

Right Ventricular Myocardial Sinusoidal–Coronary Artery Connections in Critical Pulmonary Valve Stenosis

Ventriculo-coronary arterial connections are frequently observed in pulmonary atresia with intact interventricular septum. Sustained right ventricular hypertension during cardiac morphogenesis is thought to be responsible for persistence of myocardial sinusoidal–coronary artery connections. Considering an unusual observation of ventriculo-coronary arterial connections in a patient with critical pulmonary valve stenosis, we speculate that sinusoidal connections in pulmonary atresia with intact ventricular septum occur not only because it is the only exit for blood from the blind right ventricle, but also because of intrinsic altered morphogenesis of the right ventricle myocardium.     

Anomalous Origin of Coronary Artery in Association with Aorticopulmonary Window

Aorticopulmonary window associated with anomalous origin of coronary arteries is rare; only 12 cases have been reported previously. Origin of coronary arteries from the communicating bridge is rare, having occurred in only five of these cases. We describe two additional cases of this entity, in one of which the right coronary artery arose from the window proper. We also review the previously reported cases.     

A Direct Comparison of Right and Left Ventricular Performance in the Isolated Neonatal Pig Heart

A comparison is presented between the performance of the right ventricle (RV) and the left ventricle (LV) in neonatal hearts studied under conditions of volume loading and tachycardia. Isolated, atrially paced (150 or 300 bpm), isovolumically beating pig hearts (1–3 days of age) underwent retrograde aortic perfusion with a nonrecirculating, crystalloid solution. Ventricular pressure was assessed with saline-filled balloon catheters, which allowed separate loading of the RV or LV. Both ventricles showed an initial increase followed by a leveling off, but no decline, in peak systolic pressure (PSP) and +dP/dt _max with volume loading up to an end-diastolic pressure (EDP) of 18 mmHg. The LV generated a higher PSP and +dP/dt _max compared to the RV at equivalent pressure or volume preloads. However, the maximal systolic elastance (E _max) was comparable for both ventricles. Although the RV demonstrated a greater compliance than the LV, the myocardial relaxation time constant (τ) was similar for both chambers at equivalent volume preloads (sarcomere stretch). Positive dP/dt _max correlated closely and in the same linear fashion with −dP/dt _max for both ventricles, indicating that the RV and LV exhibited similar contraction–relaxation coupling. Increasing the heart rate to 300 bpm decreased PSP, +dP/dt _max, and −dP/dt _max and increased EDP for both ventricles, whereas E _max and τ were not significantly altered. Thus, although there are differences between the functional properties of the neonatal RV and LV, there are also important similarities, especially with regard to myocardial relaxation.     

Dobutamine Stress Echocardiography in Anomalous Left Coronary Artery

The evaluation of left ventricular function with dobutamine stress echocardiography is described for the first time in a patient with anomalous left coronary artery from the pulmonary trunk during the preoperative and postoperative periods. This method demonstrated signs of myocardial ischemia that were not seen on the resting echocardiogram during the preoperative period and ventricular function recovery after surgical intervention.     

Right Aortic Arch with Retroesophageal Left Aberrant Innominate Artery

Right aortic arch with a left retroesophageal innominate artery (type D double aortic arch) is rare. The diagnosis is made by aortography. The present case is the first known patient to undergo a magnetic resonance imaging study that outlined the anomaly clearly.     

Successful Embolization of Coronary Arteriovenous Fistula Using an Interlocking Detachable Coil

Although the efficacy and feasibility of coil embolization of coronary arteriovenous fistulas have been reported, the procedure may be complicated by migration of the coil into peripheral vessels or pulmonary arteries. We report two cases of successful coil embolization of such lesions using an interlocking detachable coil. This system can provide safer and more effective coil embolization in patients with coronary arteriovenous fistula.     

Recurrent Congenital Left Atrial Aneurysm in a Newborn

Recurrent congenital left atrial aneurysm presenting in a newborn as cardiac tamponade is described. Such a presentation has not been previously reported.     

Transcatheter Occlusion of a Large Left Coronary Artery to Right Superior Vena Cava Fistula Using the Amplatzer Duct Occluder Device

Transcatheter embolization has become the therapy of choice for most coronary artery fistula. We report a 5.9-kg infant with a coronary artery fistula from the left coronary artery to the right superior vena cava with significant congestive heart failure. The infant underwent successful complete occlusion using the Amplatzer duct occluder.     

Ventricular Arrhythmia Associated with Respiratory Syncytial Viral Infection

Respiratory syncytial virus (RSV) is a common cause of lower respiratory infections in infants. Cardiac arrhythmias are an uncommon complication in infants with RSV bronchiolitis. Presumably, the arrhythmias may be secondary to RSV pericarditis, myocarditis, or some other infection of the heart. We report on a patient with RSV bronchiolitis who had clinical myocarditis complicated by ventricular tachycardia.     

Course of Right and Left Ventricular Function in Patients with Pulmonary Insufficiency After Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 ± 0.10) and left ventricular ejection fraction (LVEF; 0.68 ± 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 ± 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 ± 0.11 (p < 0.02). This change was independent of the RVEF (r=−0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.     

Aortic Root Abscess with Coronary Artery Involvement: Diagnostic and Surgical Management

We describe two cases of infectious endocarditis in infants complicated by aortic root abscess involving the coronary artery treated by the Ross operation. Multislice computed tomography was important in defining the diagnosis and in planning subsequent surgical management.