Otto G. Thilenius

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.

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.

Advantages of early relief of subaortic stenosis in single ventricle equivalents

Thirteen patients with single ventricle equivalents and subaortic stenosis underwent relief of the stenosis and subsequent Fontan operation. Nine patients, group 1, had the obstruction relieved at 3.6 +/- 1.6 years of age whenever the pressure gradient became apparent. Four patients, group 2, had the subaortic stenosis operated on at the neonatal period, 10.5 +/- 10 days old, before hemodynamic evidence of obstruction. Preoperative pressure gradient across the outflow tract was 44.2 +/- 4.7 mm Hg in group 1 versus 4.7 +/- 5 mm Hg in group 2 (p = 0.002). Ventricular muscle mass was 186% +/- 18% in group 1 versus 114% +/- 5% of normal in group 2 (p = 0.0001), and mass/volume ratio was 1.12 +/- 0.62 in group 1 versus 0.62 +/- 0.16 in group 2 (p = 0.003). Relief of subaortic stenosis was achieved by proximal pulmonary artery to ascending aorta or aortic arch anastomosis and by systemic to distal pulmonary artery shunt. There was no hospital mortality or complication related to the procedure. At evaluation before Fontan operation, 4.3 +/- 1.6 years after relief of subaortic stenosis in group 1 and 3.2 +/- 0.9 years in group 2, the pressure gradient across the ventricular outflow tract was 4 +/- 3 mm Hg in group 1 versus 3 +/- 2 mm Hg in group 2 (p = not significant), ventricular muscle mass was 184% +/- 31% in group 1 versus 114% +/- 5% of normal in group 2 (p = 0.003), and the mass/volume ratio was 1.17 +/- 0.2 in group 1 versus 0.62 +/- 0.2 in group 2 (p = 0.003).(ABSTRACT TRUNCATED AT 250 WORDS)

Angiographic Right and Left Ventricular Volume Determination in Normal Infants and Children

Right and left ventricular volumes were calculated from bi-plane cineangiograms using a previously descriped parallelepiped method. Seventeen children, 1 month-10 years of age, were studied. Mean right ventricular end-diastolic volume (EDV) was 64 ml/m2 for infants, 78 ml/m2 for children. Mean right ventricular end-systolic volume (ESV) was 30 ml/m2 for both age groups. Left ventricular EDV and ESV were smaller than their right ventricular correlates by 23% and 56%, respectively. Mean right ventricular ejection fraction was 0.52 for infants and 0.61 for children. Mean left ventricular ejection fraction was 0.79 and 0.74, respectively.Speculation Cineangiographic determination of right and left ventricular volumes from a single contrast injection provides an excellent method to simultaneously evaluate hemodynamic performance of the two ventricles. Analysis of frame to frame volume changes, coupled with exact measurements of ventricular pressure changes, may allow calculation of ventricular distensibility and contractility.

Electrophysiologic abnormalities of children with ostium secundum atrial septal defect

Sinus node (SN) and atrioventricular node (AVN) function were evaluated in 49 patients with secundum type atrial septal defect (ASD). Automaticity and conduction system function were assessed by intracardiac recording of the AH and HV intervals at rest, corrected SN recovery time, sinoatrial conduction time, AVN refractory period and the ability of the AVN to conduct rapidly paced atrial beats to the ventricles. Electrophysiologic abnormalities were found in 41% of the 34 patients who were studied before surgery. However, no preoperative abnormalities were present in children younger than 2.5 years. If only children older than 2.5 years were analyzed, the incidence of conduction abnormalities was similar for the patients studied before operation (62%) and those studied after operation (71%). The size and ejection fractions of the right and left ventricles, the magnitude of shunt flow and the size of the ASD did not differ between the patients with and those without electrophysiologic abnormalities. AVN dysfunction was present in 40% of the patients who were studied after surgical repair. While this frequency was more than twice the preoperative incidence of AVN dysfunction, it was not statistically significant. The data suggest that patient age is the major factor that influences the presence of conduction system dysfunction in patients with ASD.

Friedreich's ataxia associated with idiopathic hypertrophic subaortic stenosis

Abstract Friedreichs ataxia and idiopathic hypertrophic subaortic stenosis (IHSS), both diseases of unknown etiology, both result in hypertrophy and degenerative changes of the myocardium. Both diseases lead to nearly identical electrocardiographic changes. We report a patient who developed classical signs of Friedreichs ataxia several years after he was found to have moderately severe IHSS. This observation prompted a hemodynamic study of five other patients with Friedreichs ataxia in search of a more common association and thus a possible etiological link between the two diseases. Cardiac catheterization was performed under local anesthesia. Catheters were inserted into the right ventricle, the pulmonary artery, the left ventricle, and the aorta. Isuprel infusion up to 6 μg per minute was used in an attempt to induce an abnormal pressure gradient across the right or left ventricular outflow tract. Both right and left ventricular cineangiograms were obtained for evaluation of ventricular kinetics and wall thickness. Right and left heart pressures were normal at rest in all patients. During Isuprel infusion a 40 mm. pressure gradient was induced across the left ventricular outflow tract in only one patient. A second patient developed a 16 mm. gradient across the right ventricular outflow tract. All patients had low normal cardiac outputs. The angiograms demonstrated increased left ventricular wall thickness in all patients. It is concluded that systolic left and/or right ventricular out-flow tract obstruction is only infrequently present in Friedreichs ataxia and is mild in degree. An etiological link between Friedreichs ataxia and IHSS is not known at the present but may possibly exist and should be investigated.

Angiographic Diagnosis of an Anomalous Right Coronary Artery Arising from the Pulmonary Artery

Abnormal origin of the coronary arteries from the pulmonary artery can occur in four different ways. Either the left or the right coronary artery, both coronary arteries, or an accessory coronary artery may arise from the pulmonary artery. The most frequent of these malformations is the abnormal origin of the left coronary artery. Many publications have dealt with the clinical, physiological, and anatomical picture of this malformation (7). The majority of patients are symptomatic in early life because of marked anoxia of the anterolateral and apical portions of the left ventricle, the result of insufficient intercoronary anastomoses. Some patients, however, may become symptomatic later in life, and in these abundant anastomoses are found between both coronary arteries. From catheterization and angiographic investigations, there is conclusive evidence that the blood flow is directed from the right coronary artery through intercoronary anastomoses into the distal branches of the left coronary artery and fr...

Cellulitis of the leg due to type B Hemophilus influenzae

Summary An isolated cellulitis of the legin a 6-month-old infant was demonstrated to result from infection with a type b Hemophilus influenzae organism. Two other frequent, or rare, complications of hematogenous dissemination of this virulent pathogen in young infants such as meningitis, pyarthrosis, or osteomyelitis, were not seen in this case.

Variations in expression and treatment of transient neurocardiogenic instability

Whereas previous reports describe the head-up tilt test for the diagnosis of neurocardiogenic syncope, this study focuses on the events leading up to syncope. These events are part of a spectrum of neurocardiogenic instability; syncope is the extreme end point. This report is based on tilt studies of 108 patients, aged 4 to 22 years (mean 13), with histories of either unexplained syncope or episodes of visual blackout without loss of consciousness. The tilt study was positive in 74% of the patients. Five stages of neurocardiogenic instability were identified, ranging from excessive fluctuations in heart rate to full syncope. Uncommon reactions during the tilt study include dissociation of hypotension, bradycardia and vagal symptoms and extended asystole (greater than 6 seconds). Therapy with beta 1-selective blockers was highly successful in suppressing symptoms of neurocardiogenic instability. Repeat tilt testing with beta blockade has resulted in either normal or markedly improved stability in heart rate and blood pressure. Neurocardiogenic instability is a common condition occurring between age 10 and 20 years. It appears to be self-limited.

Kinking of the aorta (Pseudocoarctation) with coarctation

Abstract Pseudocoarctation and coarctation of the aorta in a 7 year old boy are reported. This association is very unusual, particularly in a young child. A possible pathogenetic relation between these two anomalies is discussed.