Grace C. Kung

Intracardiac Thrombi in Pediatric Patients: Presentation Profiles and Clinical Outcomes

We document the presentation profiles, treatment strategies, and clinical outcomes in a relatively large cohort of pediatric patients with intracardiac thrombi (ICT). We performed a retrospective review of patients diagnosed with ICT by echocardiography at a tertiary pediatric hospital during a 10-year period. These patients received medical therapy or thrombectomy. We provided echocardiographic descriptions of the ICT—size, chamber location, and mobility/morphology. The outcome measures were ICT (persistence, resolution, or embolization), effectiveness of therapy, and patient morbidity and mortality. There were 40 ICT diagnosed in 31 patients (22 males and 9 females). Mean age at diagnosis was 8.8 years (range, 15 days to 18 years). Overall mortality was 12/31 patients (39%); only one death was attributed to ICT embolization. Embolic events occurred in 4/31 patients (13%). The most common initial therapies included heparin infusion (n = 15), warfarin (n = 7), and aspirin (n = 7). The ICT resolved with medical therapy alone in 19/30 patients (63%). One patient required surgical thrombectomy. The cohort was divided into group 1 (dilated cardiomyopathy), group 2 (status post Fontan operation), and group 3 (other diagnoses). In group 1 (n = 11), there were 8 deaths. Embolization occurred in 2/5 large ICT, resulting in cerebral infarction and death (n = 1) and renal infarction (n = 1). The most common ICT location was the left ventricle (n = 10). Severe ventricular systolic dysfunction was present in 10/11 patients (91%). In group 2 (n = 9), there was 1 death. Embolization occurred in 1/7 large ICT, resulting in seizures and temporary paresis. All ICT were located in the Fontan pathway. Severe ventricular systolic dysfunction was present in 2/9 patients (22%). In group 3 (n = 11), there were 3 deaths. Embolization occurred in 1/9 small ICT, resulting in coronary emboli. ICT are most commonly diagnosed in pediatric patients with dilated cardiomyopathy or patients status post Fontan operation. The majority of ICT resolve with medical therapy. Larger ICT tend to embolize more frequently, and the morbidity secondary to embolizatioh is significant. Rarely is mortality due to ICT embolization. The prognosis is poor for patients with left ventricular ICT or ICT in the presence of ventricular systolic dysfunction.

Retrograde Transcatheter Coil Embolization of Congenital Coronary Artery Fistulas in Infants and Young Children

We report the success of transcatheter coil embolization of congenital coronary artery fistulas in infants and young children, primarily using a retrograde coaxial delivery system. Transcatheter coil embolization of coronary artery fistulas has been used successfully in the adult population with limited reports in the pediatric population, especially in infants. Ten patients underwent transcatheter coil embolization of congenital coronary artery fistulas between January 1993 and August 1999. The median age was 4.5 years (ranging from 5 weeks to 9.8 years). Coils were delivered exclusively using a retrograde arterial approach in 8 of 10 patients. A coaxial system was used in 8 patients. Angiography immediately after placement of coils revealed no residual shunt in any patient. Recent follow-up evaluation is available for 9 patients (median duration of follow-up, 7.9 months). Echocardiographic and clinical evaluations show complete occlusion in 8 of 9 patients. All patients are asymptomatic with normalization of chamber dimensions and coronary artery caliber. Transcatheter coil embolization of congenital coronary artery fistulas can be performed safely and successfully in infants and young children. The use of a retrograde approach and coaxial system is safe and effective and may provide better accuracy, stability and precision for placement of coils.

Hypoplastic Left Heart Syndrome in Patients with Kabuki Syndrome

The association of cardiac defects with Kabuki syndrome has been well described. The majority of these defects are isolated shunt lesions, conotruncal abnormalities, or various forms of arch obstruction. This report describes a series of three patients with hypoplastic left heart syndrome and Kabuki syndrome. The series illustrates the full spectrum of left-sided obstructive lesions and expands the phenotype of cardiac defects associated with Kabuki syndrome.

Echocardiographic Guidance for Transcatheter Coil Embolization of Congenital Coronary Arterial Fistulas in Children

Abstract. Congenital coronary arterial fistulas are rare anomalies that have traditionally been managed by surgical ligation. However, in recent years endovascular therapy has been employed with encouraging results. Between 1993 and 1996, we performed transcatheter coil embolization of coronary arterial fistulas to the right atrium or ventricle in four children ranging in age from 4.5 to 9.8 years. Cardiac and coronary arterial anatomy were diagnosed correctly on the preoperative echocardiogram in all patients, including the origin, course, and termination of the fistulas. The fistula was occluded completely in three of the patients, whereas trivial residual flow remained in the fourth. Transesophageal echocardiography was useful for monitoring the embolization procedure. In one of the patients, the fistula reopened while the child was on overnight heparin, although the magnitude of flow was less than that before the embolization. At follow-up ranging from 10 to 43 months, there was no flow through the fistula in any patient. We present our experience with these patients, with a focus on the importance of echocardiographic evaluation before, during, and after transcatheter therapy of coronary arterial fistulas.

Case Report of a 2-Year-Old Boy With Takayasu’s Arteritis: An Atypical, Severe Presentation of a Rare Disease

Takayasu’s arteritis is a granulomatous giant-cell arteritis of the aorta and its major branches. It primarily affects East Asian women in their second or third decade of life but is well known to affect all ethnicities across the world. Given its systemic nature, Takayasu’s arteritis has multiorgan involvement, with the majority of disease morbidity related to the cardiovascular, central nervous, and renal systems. This report describes an unusual presentation of Takayasu’s arteritis in a 2½-year-old boy. This is the first report in the literature describing the presence of both severe aortic regurgitation and diffuse coronary artery involvement in a patient so young.

Single-Ventricle Outcomes After Neonatal Palliation of Severe Ebstein Anomaly With Modified Starnes ProcedureClinical Perspective

Background: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. Results: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5–9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6–8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2–2.3) W/m2, and mean pulmonary pressure was 12 (9–18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. Conclusions: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.

Infected Pseudoaneurysm of a Right Modified Blalock–Taussig Shunt

V.V. Dimas, J.P. Kovalchin, E.D. McKenzie, G.C. Kung Department of Pediatrics, Lillie Frank Abercrombie Section of Pediatric Cardiology, Baylor College of Medicine, Texas Children’s Hospital, 6621 Fannin Street, MC 19345-C, Houston, TX, 77030, USA Michael E. DeBakey Department of Surgery, Division of Congenital Heart Surgery, Baylor College of Medicine, Texas Children’s Hospital, 6621 Fannin Street, Houston, TX, 77030, USA

Anomalous left coronary artery from the pulmonary artery discovered following total anomalous pulmonary venous return repair: a rare entity.

We describe a case of anomalous left coronary artery from the pulmonary artery in association with total anomalous pulmonary venous return. The infant was diagnosed with total anomalous pulmonary venous return at 6 weeks of age and underwent successful surgical repair. On routine follow-up, he was found to have an anomalous left coronary artery from the pulmonary artery without evidence of mitral regurgitation or left ventricular dysfunction. The presence of the left-to-right shunt and secondary elevation in pulmonary artery pressures likely masked the usual findings associated with this coronary anomaly.

Prenatal diagnosis of tetralogy of Fallot with a double aortic arch.

In this study, we present a case of prenatally diagnosed tetralogy of Fallot with a double aortic arch, correlating images from fetal echocardiography, transthoracic echocardiography, and cardiac MRI.

Postoperative Serum Troponin Trends in Infants Undergoing Cardiac Surgery

Troponin-I (TN-I) levels are elevated following pediatric cardiac surgery with speculation that particular patterns may have prognostic significance. There is lack of procedure-specific data regarding postoperative TN-I levels in infants undergoing cardiac surgery. We hypothesized that TN-I elevation varies with type of surgery and persistent elevation predicts poor prognosis. We prospectively measured serial TN-I levels (preoperatively, 4, 8, 12, 24, and 48 hours postoperatively) in 90 infants (age < 1 year) undergoing cardiac surgery: off cardiopulmonary bypass (CPB) (n = 15), on CPB (n = 43), and on CPB with ventricular incision (CPB with ventricular incision; n = 32). All patients had undetectable baseline TN-I levels. The area under the curve of TN-I levels over the 48-hour period was significantly different among the surgical groups (P < 0.002), and highest in patients with CPB with ventricular incision. Generally, TN-I levels peaked by 4 hours after surgery and returned to near-normal levels within 48 hours. A persistent TN-I rise beyond 8 hours after surgery was a strong predictor of postoperative hypoperfusion injury (defined as a composite endpoint of end-organ injury resulting from inadequate perfusion, odds ratio 21.5; P = 0.001) and mortality (30% in those with persistently high TN-I, compared with 3.5% in the remaining patients; P < 0.001), independent of patient age, anatomy and/or complexity of surgery, and level of postoperative support. Our data provide benchmark values for TN-I levels following cardiac surgery in infants. Extent of TN-I elevation correlates with type of surgery. Persistent TN-I elevation beyond 8 hours after surgery is strongly associated with postoperative hypoperfusion injury and mortality.