Detection of coronary artery abnormalities in tetralogy of Fallot by two-dimensional echocardiography.

Patients with tetralogy of Fallot have a 5% to 19% incidence rate of abnormal distribution of coronary arteries. These abnormalities are usually detected by angiography and influence the timing and mortality rate of surgery. This study evaluates two-dimensional echocardiography as a method of assessing coronary artery distribution in tetralogy of Fallot. Forty-five consecutive patients with tetralogy of Fallot, aged 0.1 to 20.5 years (mean 5.7 +/- 4.3), had prospective two-dimensional echocardiographic studies to examine the branching patterns of the coronary arteries and to determine the presence or absence of a branch from the right or left coronary artery that crossed the right ventricular outflow tract. The first two patients had known coronary abnormalities and served as learning models. All other echocardiographic studies were performed without knowledge of angiographic or surgical findings. Twenty-two studies were completed before coronary angiography (group A) and 23 after angiography (group B). All eight patients (18%) with coronary abnormalities were correctly identified by two-dimensional echocardiography (five in group A and three in group B). Three had bilateral anterior descending coronary arteries originating from the left and right coronary arteries, two had the anterior descending artery originating from the right coronary artery, two had a large conal branch from the right coronary artery and one had origin of both left and right coronary arteries from a single left ostium. All abnormal coronary arteries were visualized crossing the right ventricular outflow tract, whereas all 21 small conal branches from the right coronary artery were not seen in the right ventricular outflow tract.(ABSTRACT TRUNCATED AT 250 WORDS)

Successful transcatheter closure with the buttoned device of aortopulmonary window in an adult.

This case report describes a technique to close an intermediate aortopulmonary window with the use of the buttoned device in an adult.

Long-term follow-up results of balloon angioplasty of postoperative aortic recoarctation.

Immediate- and short-term follow-up results of balloon dilatation of aortic recoarctation following surgery have been well documented, but there is sparse data on long-term follow-up. During a 10-year period ending in August 1995, 33 children, aged 2 months to 14 years old, underwent balloon angioplasty of aortic recoarctation. Prior surgery included resection and end-to-end anastomosis (n = 9), subclavian flap (n = 16) or prosthetic (Dacron or Gore-Tex) patch (n = 5) angioplasty, and repair of an interrupted aortic arch (n = 3). Recoarctation developed 1 month to 14 years (mean +/- SD 29 +/- 44 months) after surgery. The indications for angioplasty were peak-to-peak systolic gradients > 20 mm Hg and systemic hypertension and/or congestive heart failure. After balloon angioplasty, the peak-to-peak systolic pressure gradient across the coarctation decreased from 48 +/- 22 to 13 +/- 15 mm Hg (p <0.01), and the size of the coarcted segment increased from 3.3 +/- 1.4 to 6.5 +/- 2.3 mm (p <0.01). Follow-up angiography and/or magnetic resonance imaging were performed in 20 children 17 +/- 12 months after angioplasty. No aneurysms were observed and improvement in the diameter of the coarcted aortic segment (9 +/- 3 mm) persisted. One- to 10-year (median 5) clinical follow-up was available in 32 children. During follow-up, 2 children required surgery to repair a long tubular isthmic narrowing. The residual gradients, determined by arm-leg systolic blood pressure difference, were 5 +/- 8 mm Hg. No patient was symptomatic and only 1 patient (3%) was hypertensive, controlled with antihypertensive medications. We conclude that balloon angioplasty of aortic recoarctation following all types of surgical repair is feasible, safe, and effective with good long-term results. We recommend balloon angioplasty as the procedure of choice in the management of postsurgical recoarctation with hypertension and/or congestive heart failure.

Coronary artery to pulmonary artery collaterals after heart transplantation.

Collaterals are described between coronary and pulmonary arteries after orthotopic heart transplantation. It is likely the collaterals developed as a result of adhesions in the pericardial space. This is a previously unrecognized complication of heart transplantation with potential clinical implications, as coronary to extracardiac artery collaterals have been shown to cause myocardial ischemia.

Arcade mitral valve and anomalous left coronary artery originating from the pulmonary artery.

The use of color Doppler interrogation significantly improves accuracy in the evaluation of coronary artery anatomy. This was demonstrated in a patient in whom the left coronary artery originated from the posterior and medial aspect of the pulmonary artery, a particularly difficult coronary anomaly to detect by 2-dimensional imaging. In addition to the coronary artery anomaly, this patient also had an arcade mitral valve.

Aberrant origin of the left coronary artery from the right aortic sinus: surgical intervention based on echocardiographic diagnosis.

An athletic 15-year-old girl with aberrant left coronary artery from the right coronary sinus, presented with syncope during exercise. Trans-thoracic echocardiography was the only imaging technique that clearly demonstrated her anomaly. The results of magnetic resonance and selective coronary angiographic imaging were inconclusive. Surgical intervention was successfully performed on the basis of the echocardiographic diagnosis.

Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography.

Coronary artery stenosis (CAS) and coronary ostial stenosis (COS) are potentially life-threatening conditions. The echocardiographic diagnosis of CAS and COS in children has not been described. We report on the transthoracic echocardiography (TTE) findings of CAS and COS in children. Six patients, aged 1 week to 12 years, with clinically confirmed COS (n = 5) and CAS (n = l) were diagnosed by TTE. Their echocardiographic findings were compared with 26 healthy control subjects of a similar age range. Left COS was associated with an aberrant left coronary artery (CA) from the contralateral aortic sinus (n = 2), an intramural left CA with d-transposition of the great vessels (n = l), and supravalvular aortic stenosis (n = l). Right COS was present in a patient with aortic valvular stenosis. Acquired left main CAS was diagnosed in the sixth patient 3 years after orthotopic heart transplantation. Coronary ostial stenosis was recognized when a color flow acceleration signal was present proximal to and extending into the coronary ostium (CO). Coronary artery stenosis was detected when a coarctated color flow stream was present within the stenosed CA segment with turbulent distal flow. These findings were not detected in the control cohort who demonstrated laminar CA and CO flow signals. All patients had increased spectral velocity in the CA distal to the stenosed segment (patients = 50 +/- 5 cm/s, controls = 24 +/- 6 cm/sec; P <.01). Delayed peak diastolic velocity seemed to indicate severe stenosis. We conclude that (1) CO acceleration signals and turbulent coarctated CA flow signals are abnormal findings in TTE coronary Doppler assessment. They indicate COS and CAS, respectively. (2) Knowledge of the normal TTE CA flow velocity patterns is essential so that abnormal velocity signals such as seen with CAS and COS can be recognized and a timely diagnosis made.

Morphometric assessment of the innominate vein in the prediction of persistent left superior vena cava.

This study sought to develop a simple echocardiographic predictor of persistent left (L) superior vena cava (SVC) in subjects with bilateral SVC. Two groups of children were studied: one with known LSVC (n = 19) and the other, a control group, without LSVC (n = 15). Both groups were of similar age (3.5 +/- 3.0, mean +/- SD vs 3.8 +/- 3.1 years; p = 0.8) and weight (14.6 +/- 7.1 vs 15 +/- 8 kg; p = 0.9). The left innominate vein was either absent (n = 11) or hypoplastic (n = 8) in the LSVC group. The ratio of the innominate vein to the innominate artery was found to be independent of age or body surface area but was significantly smaller in the LSVC group than in the control group (0.33 +/- 0.1 vs 0.97 +/- 0.1; p < 0.001). A cutoff value of 0.47 or less discriminated the LSVC group from the control subjects. Interobserver and intraobserver variations, although important, did not influence the discriminating value of the ratio in the diagnosis of LSVC. Validation of the proposed ratio in 30 consecutive prospectively studied patients with LSVC proved to be 100% sensitive in predicting LSVC. No false-positive diagnosis of LSVC was made when this principle was applied. Absent or hypoplastic left innominate vein measuring 0.47 or less of the innominate artery is an easily recognizable and reliable echocardiographic predictor of LSVC.

Transthoracic Doppler echocardiography of normally originating coronary arteries in children.

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 +/- 23 versus 24 +/- 6 cm/sec, p = 0.0004), increased diastolic flow (16 +/- 15 versus 6 +/- 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% +/- 14% versus 20% +/- 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m2 (r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight (p < 0.001, r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve.

Balloon angioplasty of long-segment aortic coarctation in the neonate.

It is generally believed that balloon angioplasty of diffuse, long-segment aortic coarctation is not effective. In this report, we describe two neonates with diffuse, long-segment coarctation in association with complex congenital heart defects in whom we were successful in effectively treating coarctation with transumbilical artery balloon angioplasty. Based on this experience, it may be concluded that balloon angioplasty of long-segment coarctation in neonates is feasible and effective, but confirmation in a larger group of patients may be necessary prior to general adoption of this concept.

Congenital coronary artery abnormalities.

Congenital coronary artery abnormalities are rare and account for approximately 0.1 to 2% of congenital heart defects. They may pose significant risk of mortality or morbidity to the patient. The pediatrician and the pediatric cardiologist should be aware of their subtle but very serious presentations and diagnostic steps to be undertaken to pinpoint the diagnosis. Prevention of serious complications from these abnormalities can be achieved by making the appropriate diagnosis and performing timely surgical intervention. This review will discuss the most common congenital coronary artery abnormalities and their management.

Fludrocortisone in the management of an infant with a single ventricle and an overly tightened pulmonary artery band.

We describe a 2-month-old female infant with a univentricular heart and overly tightened pulmonary artery band (PAB) resulting in hypercyanotic episodes. Despite adequate doses of morphine and a beta-blocker, she continued to have multiple hypercyanotic events per day. Treatment with fludrocortisone resulted in resolution of these episodes and allowed performance of a Glenn shunt at 3 half months of age. We conclude that fludrocortisone may be beneficial in the management of an overly tightened PAB until the patient is suitable for surgical intervention.

Transumbilical venous, anterograde, snare-assisted balloon aortic valvuloplasty in a neonate with critical aortic stenosis.

Balloon aortic valvuloplasty is an acceptable alternative to surgery in the treatment of critical aortic stenosis in the neonate. In this report, we describe a 1-day-old infant with critical aortic stenosis who was successfully treated with an anterograde, transumbilical venous, snare-assisted balloon aortic valvuloplasty. Based on this experience, it is suggested that the anterograde transumbilical venous approach is a feasible and effective alternative to retrograde femoral, carotid, or umbilical arterial and transfemoral venous anterograde routes for performing balloon aortic valvuloplasty in the neonate.

Anomalous origin of the left coronary artery from the pulmonary trunk: repair after diagnostic cross sectional echocardiography.

An infant with anomalous origin of the left coronary artery from the pulmonary trunk presented with congestive cardiomyopathy. Only cross sectional echocardiography gave a definitive diagnosis. The results of cardiac catheterisation and angiography were inconclusive. Surgical repair was performed successfully after the results of cross sectional echocardiography were known.

Severe left ventricular dysfunction and arrhythmias as complications of gram-positive sepsis: rapid recovery in children.

Severe left ventricular dilatation and dysfunction in children may be associated with poor outcome in as many as 45% of cases. The prognosis for children with poor left ventricular function or arrhythmias associated with septic shock may be better, however, this has been inadequately studied. We report the favorable outcome of three children who presented with Gram-positive sepsis and significant cardiovascular compromise-two with severely dilated, poorly contractile left ventricle, and one with mild left ventricular dysfunction and incessant, malignant, and rapid atrial and ventricular arrhythmias. Our experience with these patients shows that complete and rapid resolution of these complications may be achieved with aggressive therapy.

Successful treatment of a staphylococcal endocarditis vegetation with tissue plasminogen activator.

A 930 gm premature infant had Staphylococcal endocarditis with a tricuspid valvular vegetation that was unresponsive to antibiotics and not amenable to resection. Infusion of tissue plasminogen activator over a 3-day period completely lysed the vegetation. The infection cleared with continued antibiotics, and the infant recovered without sequelae.

Resolution of prostaglandin-induced cortical hyperostosis in an infant.

Prostaglandin E1 (PGE1) has been used to preserve patency of the ductus arteriosus in various forms of congenital heart disease. Long-term PGE1 therapy often leads to the roentgenographic findings of cortical hyperostosis, similar to those seen in infectious or metabolic diseases. These periosteal reactions regress after cessation of PGE1 therapy and should not prompt further diagnostic evaluation.