Rene A. Arcilla

Congestive heart failure from suspected ductal closure in utero

This is the 1st case report of a ductal closure occurring during fetal growth. The case was a spontaneous delivery in cephalic presentation from a 31-year-old gravida 3, para 3 Black woman who had been treated with isoniazid and spreptomycin up to 2 months before her delivery. Gestational age was 37 weeks when the fetus was delivered weighing 3.15 kgm. The cord had been wrapped around the fetuss neck, and breathing was delayed 2 minutes. In the nursery, the babys general condition was poor, and congestive heart failure was diagnosed. The newborn had trieuspid insufficiency, severe heart failure, and acidosis at birth. These disappeared the next day. Hemodynamic studies when the baby was 4 hours old showed a large cone-shaped ductus arteriousus extending from the pulmonary artery but ending blindly at the aortic end.

Cardiac Status in Juvenile Borderline Hypertension

A prospective M-mode echocardiographic study was done to look for early cardiovascular changes in children prone to hypertension with blood pressures between the 75th and 95th percentiles for age. Average systolic/diastolic pressures in 27 children with borderline hypertension were 137/89 mm Hg compared to 110/68 mm Hg for the 26 controls. Echocardiographic measurements were normalized for comparison using two methods. The borderline hypertensive group mean values were significantly greater than controls for left ventricular wall thickness (p less than 0.05 for method 1; p less than 0.001 for method 2), left ventricular mass (p less than 0.001; p less than 0.005) and left ventricular wall thickness to radius ratio (p less than 0.001, both methods). Echocardiographic estimates of left ventricular function were lower in the hypertensive group. This study suggests that cardiac hypertrophy can be shown by noninvasive means in some children before arterial pressure becomes elevated. To assess the incidence and possible consequences of early target organ changes, more extensive clinical evaluation of borderline hypertension in children is recommended.

Single (Primitive) Ventricle

Single ventricle is that condition in which both atrioventricular (A-V) orifices enter a common ventricular sinus, from which the arterial trunks emerge with their coni. The following types of single ventricle were found: (1) with regular (noninverted) transposition in levocardia; (2) with inverted transposition in levocardia; (3) with normal position of arterial trunks in levocardia; (4) in dextrocardia, and (5) in mesocardia. This entity is differentiated from common ventricle which is basically a heart with a large ventricular septal defect. Single ventricle does not include mitral and tricuspid atresia by definition, nor does it include straddling tricuspid orifice, in which two distinct ventricles are present. The concept of single ventricle herein defined differs from de la Cruz and Millers concept of double-inlet left ventricle which includes straddling tricuspid orifice. Embryologically in single ventricle we are dealing with persistence of the primitive state of the bulboventricular loop, due to a lack of expansion of the atrial canal to the right during the process of absorption of the bulbus.

Regression of myocardial hypertrophy. I. Experimental model, changes in heart weight, nucleic acids and collagen.

Left ventricular hypertrophy was induced by placing a constricting silver band around the ascending aorta in rats. These bands were then removed either 10 days (early debanding) or 28 days (late debanding) after banding. After early debanding the left ventricular mass decreased rapidly from 35% to 11% above the control values (P < 0.001) 3 days post-debanding. Left ventricular weights of the debanded and control groups were not statistically different 7 to 28 days after debanding except at 10 days. The left ventricular RNA of the debanded animals fell from 37% to 19% above the control group 3 days after surgery (P < 0.05), and remained at control levels for the rest of the study period. The left ventricular DNA of the debanded group, however, remained elevated; i.e., 32% above control levels (P < 0.001), 10 days post-debanding, and 12% at 28 days (P = n.s.). Left ventricular hydroxyproline levels of the debanded group at these times were 90% (P < 0.001) and 80% (P < 0.025), respectively, above those of controls. In the late debanding study, both left ventricular mass and RNA fell less than in the early study. The left ventricular mass remained significantly elevated, i.e., 12% above that of controls (P < 0.025), 21 days post-debanding, and left ventricular RNA was 34% above control values (P < 0.01) at 14 days. Both decreased to control values thereafter. Left ventricular DNA and hydroxyproline content did not decline, remaining 25% (P < 0.02) and 118% (P < 0.001), respectively, above the control values 28 days post debanding. The data show that while left ventricular mass and RNA content decrease after relief of a pressure overload, left ventricular DNA and hydroxyproline content does not.

Premature narrowing or closure of the foramen ovale

P remature narrowing or closure of the foramen ovale is an infrequent but not rare anomaly. We have found 25 cases reported in the literature, the details of which are presented in Table I.1-21 Analysis of 1,150 cases of congenital heart disease studied at the Congenital Heart Disease Research and Training Center revealed 10 examples of this anomaly. This is a report of the pathologic anatomy in these 10 cases as studied quantitatively by a method previously reported.22 In addition, this is a clinical-pathologic study of one case in which the diagnosis was strongly suspected clinically.

Intermediate results of the anatomic repair for congenitally corrected transposition

BACKGROUND Anatomic repair of congenitally corrected transposition of the great arteries has several advantages over the traditional approach but lacks long-term evaluation. METHODS The data on 12 patients who had the procedure between January 1989 and June 2000 were retrospectively reviewed. Associated lesions included ventricular septal defect in 12 patients, pulmonary stenosis in 10 patients, and moderate to severe tricuspid valve regurgitation in 4 patients. Mean age at operation was 9+/-3.6 months. All patients had venous switch Mustard procedure. Tunneling of the morphologic left ventricle through the ventricular septal defect to the aorta with insertion of right ventricular to pulmonary artery conduit was performed in 10 patients, and arterial switch operation in 2. Concomitant tricuspid valvuloplasty was done in 2 patients and ventricular septal defect enlargement in 1. RESULTS There was one hospital death (9%) in the patient who needed ventricular septal defect enlargement. Complications included atrioventricular block requiring pacemaker insertion in 1 patient (9%) and superior vena caval obstruction in 1 patient (9%). Follow-up is available on all patients 0.5 to 10 years (mean, 7.6+/-3.1 years). All patients are asymptomatic. Exercise test results on the three oldest patients were normal. Bradytachyarrhythmias developed in 4 patients (36%). Right ventricular to pulmonary artery conduit replacement was needed in 5 patients 2.2 to 7.1 years (mean 5.2+/-3.6 years) postoperatively. Mild to moderate tricuspid valve regurgitation persisted in 2 patients. Systemic left ventricular fractional shortening was 36% to 47% (mean, 39%+/-4.6%), and ejection fraction was 49% to 70% (mean, 60.8%+/-7.9%). CONCLUSIONS The double switch operation can be performed safely with minimal intermediate and long-term complications.

Echocardiographic functions and blood pressure levels in children and young adults from a biracial population: The bogalusa heart study

M-mode echocardiograms were obtained on 651 healthy subjects, 7-22 years of age, whose diastolic blood pressure levels remained in the same height-, race-, and sex-specific decile during two biannual examinations. Echocardiographic measures of heart size and dynamics were compared across the total blood pressure distribution. Left ventricular stroke volume, cardiac output and ejection fraction, minor axis shortening, velocity of circumferential fiber shortening, and peripheral vascular resistance were correlated with blood pressure levels. There were positive correlations (p less than .001) of cardiac output and stroke volume with both systolic and diastolic blood pressure levels. Left ventricular output and stroke volume were associated with measures of body size, especially height, weight, ponderal index, and body surface area (p less than .001). The left ventricular output and stroke volume increased with age and with systolic blood pressure quintiles in the four race-sex groups. With adjustment for systolic blood pressure and measures of body size, white males had greater cardiac output (1.25 l/minute for ages 18-22 years, p = .01) and stroke volume than black males. Black males had higher peripheral resistance (4.5 mm Hg/(l/minute), p = .01) than whites. These results suggest that different hemodynamic mechanisms operate in the early phase of hypertension in blacks vs. whites in this population.

Spectrum of pulmonary sequestration: Association with anomalous pulmonary venous drainage in infants

SummaryPulmonary sequestration is a spectrum of related lesions, each of which may be absent or present: (1) bronchial sequestration of pulmonary parenchyma; (2) arterial supply from systemic circulation; (3) anomalous pulmonary venous drainage to the right atrium; (4) communications between bronchus and esophagus; (5) defects of diaphragm; (6) gross lung anomalies, such as horseshoe lungs or hypoplasia. Any combination of these primary lesions can occur in an individual patient. Diagnosis should be directed towards each component of the spectrum. Of special importance is the venous connection, as anomalous pulmonary venous drainage can involve not only the sequestered segment but the entire ipsilateral lung, making surgical therapy far more complex. Treatment of choice is surgical resection, associated, if needed, with rerouting of the pulmonary venous return.Classification of sequestration of the lung as intra- and extralobar is of secondary importance: these 2 groups do not represent lesions of different embryological significance.

Straddling and Displaced Atrioventricular Orifices and Valves with Primitive Ventricles

Fourteen cases of straddling or displaced atrioventricular (A-V) orifices and valves are presented. There are three types: (1) straddling tricuspid orifice entering both primitive ventricles with the mitral valve entering the left ventricle (nine cases), (2) displaced tricuspid orifice into the primitive left ventricle, with no A-V orifice in the primitive right ventricle (three cases), and (3) displaced tricuspid orifice with straddling mitral valve, in which the mitral valve has connections in both primitive ventricles, with the tricuspid entering the primitive left ventricle (two cases). Pathologically, these hearts are not single ventricle since there is a primitive right ventricle containing a portion of sinus as well as conus. Embryologically, however, these hearts are related to single ventricle, all being due to insufficient or no passage of the atrial canal region to the right during the process of absorption of the bulbus. Selective angiocardiography may differentiate straddling A-V orifices from single ventricle by showing immediate filling of both ventricles by injection into the right or left atrium.

Pathologic Anatomy of Dextrocardia and Its Clinical Implications

This is a morphologic study of 41 cases of dextrocardia for the purpose of clarifying terminology and evaluating the clinical import of the morphologic data. Dextrocardias are classified as dextroversion, mirror-image dextrocardia, and mixed dextrocardia. When the atrial septum does not identify the atria, it was found possible to make the presumptive diagnosis of dextroversion or mirror-image dextrocardia. The mutual relations of the visceral situs with the atrial situs, the presence or absence of splenic abnormalities, the type of dextrocardia, and the systemic and pulmonic venous return were investigated, as well as the type of complex that is characteristic of each type of dextrocardia. The most important morphologic data useful for clinical application were found to be the position of the aortic and pulmonic annuli, the course of the pulmonary trunk, the entry of the inferior and superior venae cavae, and the course of the anterior descending coronary artery.