Saadeh B. Jureidini

Clinical profile of conjestive cardiomyopathy in children

Abstract The clinical profile of 23 children with congestive cardiomyopathy was reviewed to detect any factors that might be predictive for their survival. Factors examined include age at onset ( 2 years), geader, severity of the clinical picture including data from the chest radiograph electrocardiogram (ECG), echocardiogram, hemedynamic study and endomyocardial biopsy. Follow-up study ranged from 1 month to 14 years (mean 43 months). There were 12 survivors and 11 nonsurvivors; the 1 year mortality rate was 30% (7 of 23), and the 5 year mortality rate was 44% (10 of 23). Age at onset, gender, cardiothoracic ratio on chest radiograph, pattern of infarction, ST-T changes or arrhythmia on ECG and left ventricular enddiastolic pressure were nonpredictive of outcome. However, low shortening fraction (mean 11.5% in nonsurvivors versus 20.9% in survivors, p

Clinical spectrum of restrictive cardiomyopathy in children

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

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.

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)

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. Cathet Cardiovasc Intervent 2003;58:130–134.

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.

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.

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.

Severe Left Ventricular Dysfunction and Arrhythmias as Complications of Gram-Positive Sepsis: Rapid Recovery in Children

Abstract. 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.

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 Childrens 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.