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.

Access to the native atria following conduit total cavopulmonary anastomosis.

OBJECTIVES: We describe the use of trans-thoracic and trans-conduit puncture to access the atria and perform interventional procedures in patients who have undergone conduit total cavopulmonary anastomosis. BACKGROUND: Catheter access to the atria following intra or extra-cardiac Fontan is desirable when there is a need for trans-atrial interventions. METHODS: Between 2009 and 2014, 5 patients ages 7 to 28 years underwent this approach; three trans-thoracic and 2 trans-conduit punctures. Various therapeutic aims were achieved. Included are: placement of pacing wire in the left atrial appendage, access to re-canalized left superior vena cava via the coronary sinus for device occlusion eliminating cyanosis, access with subsequent device closure of a dormant pulmonary valve thought to be the nidus of an embolic event, and access to the atria for ablation of an atrial tachycardia. RESULTS: Entry to the atria was successful in all five patients with either trans-thoracic access or trans-conduit puncture with subsequent intended intervention performed successfully. CONCLUSIONS: Trans-conduit puncture and trans-thoracic access may allow therapeutic procedures which mitigate the need for further open heart surgery. © 2017 Wiley Periodicals, Inc.

Arrhythmias in a long-term adult survivor with uncorrected tetralogy of Fallot: Case report and review of the literature.

We present a case of a middle-aged adult with uncorrected Tetralogy of Fallot (TOF) with pulmonary atresia who developed symptomatic supraventricular and ventricular arrhythmias. The lack of data regarding management of electrical and other complications in adults with uncorrected TOF is highlighted and emphasizes the need for a registry to better understand the medical management of long-standing adult survivors.

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.

Hybrid versus Norwood procedure for hypoplastic left heart syndrome: contemporary series from a single center.

OBJECTIVE: Two different strategies have emerged in the initial palliation for hypoplastic left heart syndrome, the conventional Norwood operation and the so-called hybrid procedure. We have used each of these at our center. The purpose of the present study was to compare the outcomes of both procedures. METHODS: From 2007 to 2012, 40 patients presented to the Cardinal Glennon Children's Medical Center with hypoplastic left heart syndrome or 1 of its variants. Of the 40 patients, 24 underwent a hybrid procedure and 16 a Norwood procedure for initial palliation. The medical records, echocardiograms, and cardiac catheterization data were retrospectively reviewed. Standard statistical analysis was performed. RESULTS: The patients who underwent the hybrid procedure weighed less than those who underwent the Norwood procedure. Overall unadjusted survival was better in the Norwood group, although this did not reach statistical significance. Overall hospital resource usage was similar in both cohorts, taking into account both first and second palliation stages. CONCLUSIONS: In our review, we found no statistically significant difference in survival or resource usage between those patients undergoing the Norwood procedure and those undergoing a hybrid procedure as initial palliation for hypoplastic left heart syndrome.

Partial anomalous pulmonary venous return: transcatheter repair.

This report describes a case of partial anomalous pulmonary venous return in a young white girl for whom transcatheter intervention successfully replaced surgical correction. This intervention was feasible because the left lung had dual pulmonary venous drainage.

A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit.

BACKGROUND: This study compared the modified Blalock-Taussig (MBT) shunt with the right ventricle-to-pulmonary artery (RVPA) conduit with respect to outcome and PA growth. METHODS: PA growth was assessed in 19 MBT patients and in 15 RVPA patients before stage 2 palliation for hypoplastic left heart syndrome. The RVPA was done with a ringed Gore-Tex tube (W. L. Gore and Assoc, Flagstaff, AZ) at each anastomosis. RESULTS: The two cohorts had similar pre-Glenn demographic and hemodynamic data. No patient required transcatheter or surgical intervention on the shunt or PAs after stage 1 palliation. The branch PA growth was better in RVPA (McGoon ratio: MBT, 1.5±0.2 vs RVPA, 2.0±0.6; p<0.003) and was significantly more balanced (right-to-left PA area ratio: MBT, 1.5±0.5 vs RVPA, 0.9±0.6; p=0.002). The Nakata index trended higher in RVPA (MBT, 242A±90 mm2/m2 vs RVPA, 267±95 mm2/m2, p=0.2). After stage 2 palliation, oxygen saturation trended higher in the RVPA (81%±5%) vs MBT cohort (77%±8%, p<0.08). CONCLUSIONS: The Norwood operation using a RVPA nonvalved conduit is associated with improved branch PA growth.

Hybrid procedure for obstructive neonatal left ventricular tumors.

We report the use of the hybrid procedure (bilateral pulmonary artery banding, ductal stenting followed by balloon atrial septostomy) in the first week of life for the treatment of two neonates with large left ventricular fibromas resulting in univentricular physiology. At 6 months of age, second-stage palliation was accomplished and the 2 patients are growing well with normal developmental milestones and no atrial or ventricular ectopy at 30 months. Hybrid strategy offers a less invasive, initial intervention and the options of typical second-stage univentricular palliation, conversion to biventricular physiology in cases of tumor regression, planned surgical resection, or transplantation.

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.

Stents in the successful management of protein-losing enteropathy after fontan.

A 5-year-old female presented with anasarca secondary to protein-losing enteropathy after fenestrated extracardiac Fontan. There was no response to digoxin, furosemide, spironolactone and captopril. She had coarctation of the aorta and left pulmonary artery stenosis resistant to multiple surgical and balloon interventions. Stent expansion of these lesions resulted in the patient's recovery from protein-losing enteropathy.

An alternative method to fenestrate the extracardiac fontan conduit in a patient with atrial situs inversus and levocardia.

This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.

Comparison of angioplasty and surgery for neonatal aortic coarctation.

BACKGROUND: The efficacy of balloon dilatation as primary treatment for neonatal aortic coarctation remains controversial. METHODS: A retrospective comparison between balloon angioplasty and surgery for the treatment of neonatal aortic coarctation was undertaken on 57 neonates younger than 40 days of age (angioplasty, 23 patients; surgery, 34 patients) treated between 1994 and 2004. RESULTS: Cohorts were similar with respect to the preinterventional variables of age, weight, upper extremity systolic blood pressure, coarctation gradient, degree of aortic arch hypoplasia, associated conditions, and mean follow-up (angioplasty, 36 months; surgery, 38 months). Among the angioplasty group, 13 patients (57%) required surgery, and 8 required a second balloon dilatation, of whom 3 patients had an aortic aneurysm. Among the surgery cohort, 6 patients experienced recurrence (18%) after either SFA (3) or XETE anastomosis repair (3). All were successfully treated with balloon angioplasty. Actuarial freedom from any intervention was significantly greater in the surgery cohort as was the degree of aortic arch growth. At latest follow-up, antihypertensive medication was required in 3 of 9 angioplasty patients (33%) and 2 of 27 surgery patients (7%). No repeat intervention was required in the 13 patients who underwent angioplasty followed by surgery. CONCLUSIONS: Primary angioplasty is palliative treatment for neonatal aortic coarctation, but it is the treatment of choice for recurrence after surgery. Surgery for neonatal aortic coarctation is associated with fewer reinterventions, improved aortic arch growth, no aortic aneurysm formation, and decreased need for antihypertensive medication when compared with neonates treated primarily with balloon angioplasty.

Cardiac surgery in the newborn: improved results in the current era.

Pediatric cardiovascular surgeons can now safely operate on newborns to palliate or completely correct congenital heart defects in babies as small as 1.5-2.0 kilograms. The various cardiac anomalies treated can be categorized with respect to their dependency on a patent ductus arteriosus for systemic or pulmonary blood flow. The early results are directly linked to the complexity of the congenital defect, but are generally good so long as the intervention can provide two functional ventricular chambers. Although great strides have been made, continued progress in the treatment of neonates with congenital heart defects remains challenging and requires the collaborative effort between pediatricians, perinatologists, neonatologists, interventional cardiologists, pediatric cardiovascular surgeons and intensive care unit nurses.

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.