Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.

Bacterial pancarditis with myocardial abscess: successful surgical intervention in a 14-month-old boy.

A 14-month-old boy with a structurally normal heart presented with signs of cardiac tamponade caused by purulent pericarditis. During his hospital stay, mitral and tricuspid valve endocarditis developed, and a ventricular septal abscess expanded despite appropriate, prolonged antibiotic therapy for methicillin-resistant Staphylococcus aureus. The day before scheduled surgical correction, the abscess ruptured, creating a septal aneurysm. Surgical intervention resulted in an excellent outcome. Throughout the patient's 67-day hospitalization, the use of echocardiography was crucial in monitoring and diagnosis. In addition to reporting this case, we discuss our diagnostic and treatment considerations. To our knowledge, this is only the 4th report of S. aureus bacterial pancarditis with myocardial abscess.

The fantastic journey of a bullet: out with a snare.

Bullet embolus is a rare complication of penetrating missile trauma. Removal of the bullet previously required surgery. We report the case of a 14-year-old with an hepatic vein bullet embolus following a gunshot wound to the left buttock. A transjugular approach was used to extract the bullet percutaneously with an Amplatzer gooseneck snare.

Aberrant coronary arteries: a reliable echocardiographic screening method.

BACKGROUND: Aberrant origin (ABO) of a coronary artery (CA) from the contralateral aortic sinus with a subsequent interarterial course is a life-threatening condition. It carries a 28% to 55% risk for a sudden coronary event or death, but there are no reliable screening methods. OBJECTIVE: We sought to determine whether imaging a cross section of a coronary segment in the anterior aortic wall on the long-axis view may be used as an echocardiographic screening sign for ABO CA. METHODS: The echocardiograms of all patients with ABO CA were evaluated for the screening sign and compared with those of age-matched control patients. RESULTS: Between January 1989 and October 2002, we identified 8 patients with ABO CA (median age: 15 years). Of these patients, 4 were symptomatic and 4 were discovered incidentally. The electrocardiogram produced normal findings in 5 of 8 patients, maximal stress test produced normal findings in 5 of 6 patients, and thallium perfusion test produced negative results in 2 of 3 patients. There were 4 patients with ABO in the main left CA, 3 with ABO in the right CA, and 1 with ABO in the circumflex CA branch. The screening sign was readily visible in 7 of the 8 patients (88%), and all 7 of these patients had ABO in a main CA. Only in the ABO in the circumflex CA branch was the screening sign not detected. The 1743 control patients (median age: 14 years) showed normal anterior aortic wall, which was void of any CA segment. In 5 control patients (0.3%, P <.001) the normal right CA was visible on the long-axis view, but was not confused for a false-positive screening sign. CONCLUSION: We concluded that the proposed screening sign for ABO CA is reliable and easily recognizable, and should prompt a comprehensive assessment of the CA.

Severe aortic coarctation in infants less than 3 months: successful palliation by balloon angioplasty.

The optimal management strategy of the neonate and young infant with native aortic coarctation (AC) is controversial. We reviewed our experience with balloon angioplasty (BA) in neonates and infants 3 months to test our thesis that BA provides successful palliation, defined as avoidance of surgery for 4 weeks along with control of presenting symptoms. We also compared the results of the transumbilical arterial (UA), transfemoral arterial (FA) and transfemoral venous anterograde (FVA) approaches we have used to accomplish BA. During a 6.5-year period ending June 2001, fifty-one neonates and infants 3 months presenting with heart failure, hypertension or both underwent UA (n = 16), FA (n = 26) and FVA (n = 9) balloon coarctation angioplasty. Immediate and follow-up results were evaluated. Acute reduction of peak-to-peak gradients across the coarctation (40 17 mmHg vs. 5 6 mmHg; p < 0.001), increase in diameter of the coarcted segment (2.2 0.5 mm vs. 5.6 0.8 mm; p < 0.001) and improvement in symptomatology occurred following BA. Surgical relief of aortic obstruction was required in 4 infants at 5, 21, 24 and 28 days after the procedure. Effective palliation was thus achieved in the remaining 47 infants (92%). During intermediate-term follow-up, twenty-two infants (50%) developed recoarctation requiring repeat balloon (n = 14) or surgical (n = 8) intervention 2 10 months (median, 3 months) after initial BA. The indication for reintervention was hypertension in all patients. At a median follow-up of 3 years (range, 0.5 5.5 years), blood pressures remained low (98 11 mmHg) with an arm/leg blood pressure gradient of 4 6 mmHg. Comparison of the groups revealed similar effectiveness both immediately and at follow-up. However, femoral artery complications were seen in only the FA group. Based on these data, we conclude that effective palliation is achieved with BA in all 3 groups, femoral artery complications are seen only in the FA group and BA is an excellent alternative to surgical intervention in the management of native AC in neonates and young infants.

Transcatheter occlusion of ruptured sinus of valsalva aneurysm: innovative use of available technology.

A 9-year-old boy was found to have ruptured sinus of Valsalva aneurysm (RSVA) and aortic coarctation. Following relief of aortic coarctation by balloon angioplasty, transcatheter coil occlusion of the RSVA was performed successfully under transesophageal echocardiographic and fluoroscopic monitoring; bioptome-assisted delivery of 0.052" Gianturco coil was undertaken via a 7 Fr sheath stabilized by an 0.035" guidewire passing through the RSVA and the sheath. This report details the technique of occlusion.

Congenital Coronary Artery Abnormalities in Children.

After a significant coronary artery abnormality is recognized in a pediatric patient, surgery or appropriate transcatheter intervention should be performed. The risk of fatality from a congenital coronary abnormality far outweighs the small risks of surgical or transcatheter intervention. Angiography, although considered the state-of-the-art method of diagnosis, has significant spatial limitations and is not always diagnostic of aberrant coronary origins from the contralateral aortic sinus. In the hands of an experienced coronary imager, color flow Doppler echocardiography is one of the best diagnostic tools for congenital coronary abnormalities. Symptoms of a coronary abnormality vary from none to a sudden coronary event that may result in death. Awareness of subtle as well as obvious symptoms is essential for a timely intervention. Surgical or transcatheter intervention in an asymptomatic child with a coronary abnormality is controversial, but it is becoming more acceptable due to a better understanding of the risks involved in unrepaired congenital coronary abnormalities. Surgical reimplantation is the treatment of choice for a patients with a pulmonary origin of a coronary artery. Surgical unroofing of the intramural segment is preferable in an aberrant coronary origin from the contralateral aortic sinus. Surgical enlargement of a stenotic ostium is recommended for ostial stenosis. Transcatheter coil embolization is becoming the treatment of choice of large coronary artery fistula.