Long-term follow-up of patients after valvotomy for congenital valvular aortic stenosis in children: a clinical and actuarial follow-up.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to R D Rowe.
Explore the source record for details and available documents.
A 14-year-old boy with tricuspid atresia, transposition of the great arteries, and previous pulmonary artery banding underwent repair by arterial switch procedure with coronary arterial reimplantation, plus connection of the right atrium and ventricle with a valved conduit. Pre- and postoperative hemodynamic and angiographic investigations are presented with comments on operative technique and the rationale for this unique operation.
Explore the source record for details and available documents.
While contrast echocardiography has been shown to delineate intracardiac shunts, no information is available concerning the sensitivity of the method. This study establishes the relative sensitivity of the technique in detecting and localizing shunts by comparing the contrast echocardiograms with the simultaneously recorded cardiogreen dye curves in 26 children during cardiac catheterization. The echocardiograms substantiated isolated intracardiac shunting as small as 5%. There were no false-positive or false-negative echocardiograms in patients with or without shunt defects. This report demonstrates the contrast echocardiographic technique to be extremely sensitive in detecting intracardiac communications. It reviews the diagnostic patterns necessary for diagnosis and proposes the technique as a sensitive, safe, and accurate alternate method of study in the cardiac catheterization laboratory.
Paired right and left atrial indicator dilution curves performed in a series of 17 patients with D-transposition of the great arteries (D-TGA) were reviewed. Taken together, the patterns obtained from patients who had trivial mixing between the pulmonary and systemic circulations were distinctive and easily recognizable. In the presence of substantial degrees of intracardiac mixing, however, the paired curves recorded become virtually identical and cannot be distinguished from curves recorded from patients with other forms of cyanotic congenital heart disease characterized by obligatory admixture. Thus paired atrial dye curves demonstrate a high degree of diagnostic specificity for the infant with D-TGA at greatest risk, normally those with a small intracardiac shunt. Such a procedure should facilitate early balloon atrial septostomy and obviate deterioration in the infant's condition due to prolonged catheter manipulation and/or angiography.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The association of either mitral or tricuspid stenosis with endocardial cushion defects has been well recognized pathologically but is infrequenctly diagnosed clinically. M mode echocardiographic features such as markedly disproportionate ventricular size, abnormal mitral or tricuspid diastolic echoes or failure to define adequately an atrioventricular (A-V) valve are strongly suggestive of associated stenosis of that valve. In this study mitral stenosis in association with endocardial cushion defect was correctly diagnosed in four of five patients. In the fifth patient (the first seen) the condition was easily diagnosed retrospectively. Tricuspid stenosis was similarly diagnosed prospectively in three of four patients. Significant stenosis was not overlooked or misdiagnosed in any patient with an endocardial cushion defect. Significant stenosis of an A-V valve in association with endocardial cushion defect carries a high mortality. It is important to recognize the combined lesion clinically because infants with it are not suitable for total surgical correction. Palliative surgical procedures with aggressive medical therapy probably offer the infant the best chance of surviving until ventricular growth may render corrective surgery feasible.
Explore the source record for details and available documents.
Five term and two premature newborn infants were referred for respiratory distress and congestive heart failure, and were found to have electrocardiographic Q or ST-T abnormalities suggesting ischemia. Echocardiographic and/or hemodynamic assessment excluded anatomic heart disease in six infants. In three infants, moderate or severe hemodynamic impairment within 36 hours of age was suggested by these studies. Myocardial perfusion images in all patients showed very poor myocardial uptake of thallium 201, compatible with global myocardial ischemia. Infants of similar age with myocarditis, or with congenital heart disease and congestive failure, had normal myocardial uptake. Rapid clinical improvement occurred within three to seven days. Two to five months later, all infants were well. Two had persistent electrocardiographic abnormalities but repeat thallium 201 imaging in six demonstrated almost normal myocardial uptake. These data provide further evidence that perinatal respiratory distress may be associated with myocardial dysfunction and congestive heart failure in some infants without anatomic heart disease, and suggest that myocardial dysfunction in these infants is associated with global myocardial ischemia, most of which is transient. The timing and nature of the insult causing the ischemia are unclear.
The earlier detection and investigation of babies with congenital structural heart disease has resulted in earlier treatment and better management of these patients. In 1965, at the Hospital for Sick Children, Toronto, 121 cases were assessed and treated; by 1975 this figure had risen to 226 cases. Few changes were noted in the incidence of the 10 most common malformations, except for the obvious increase in the incidence of patient ductus arteriosus due to survival of increasingly premature infants. The sick cardiac neonate was referred earlier in 1975, studied with newer non-invasive investigations, and, according to the severity of symptoms and signs, was sent sooner to cardiac catheterisation. 80% of babies presenting with cyanosis survived the first month and 60% of babies with congestive heart failure survived. With better surgery and perioperative care, the survival rate in the first month after surgery rose from 37% in 1965 to 70% in 1975.
The natural history of small ventricular septal defect (VSD) was studied in 50 infants for up to 10.5 years. The VSD closed spontaneously in 34 patients. Life-table analysis of the data showed that by 10 years of age, 75% of small VSD will close spontaneously; the figure is higher for defects in the muscular septum (83%).
We have presented the unique clinical and morphological features of 3 patients with an imperforate tricuspid valve and right ventricular tensor apparatus. Thus, despite valve tissue and apparatus, there was not a perforate atrioventricular connection. This most uncommon type of tricuspid atresia' was associated in all 3 cases with a congenitally absent pulmonary valve, an underdeveloped right ventricle, and a curious distortion of the ventricular septum. Indeed, 2 of these patients demonstrated severe disproportionate ventricular septal thickening, although histopathologic examination did not substantiate those features usually associated with a hypertrophic cardiomyopathy. Rather, microscopic examination revealed a sinusoidal malformation consisting of normal myocardial cells separated by branching ethothelial-lined channels which communicated with the right ventricular cavity. In addition, gross examination of these 3 specimens revealed an abnormally persistent right venous valve in 2, which subdivided the right atrium. Finally, these cases provide further evidence that the term 'tricuspid atresia' oversimplifies the observed morphological features.
Explore the source record for details and available documents.
The outlet chamber of the human heart can show considerable variations in its morphology and topography (relationship to main ventricular chamber), and such variations in anatomy and spacial relationship may be predictive of the associated intracardiac anatomy. Although there is considerable debate about whether the position of the outlet chamber is indicative of the type of bulboventricular loop (whether D- or L-), a right-sided and anterior outlet chamber has different implications than a left-sided, superior, and more posterior positioned one. The inflows into the outlet chamber can vary anatomically, and progressive changes in the calibers of these communications can adversely alter the natural history. One or both of the greak vessels, or neither, or a persistent truncus arteriosus can originate from the outlet chamber, and any significant morphological change in the outlet chamber may or may not adversely affect either the pulmonary or systemic blood supply, or both. Finally, any consideration of the outlet chamber raises important conceptual difference in terminologies. At present, there is not unanimity as to what constitutes either an outlet chamber (as compared to a "small right ventricle" in tricuspid atresia) or a single (primitive) ventricle. Any discussion of the morphological and topographical variations of the outlet chamber must be viewed with respect to the terminology employed.
Stenosis of the semilunar valve in the presence of a functionally single arterial trunk is uncommon. Three patients with truncus arteriosus, two with tetralogy of Fallot and pulmonary atresia and one with pulmonary atresia and intact septum were diagnosed as having stenosis of the truncal or aortic valve on the basis of clinical, echocardiographic, hemodynamic and angiocardiographic findings. Echocardiograms consistently showed multiple diastolic closure lines and abnormal semilunar valves in addition to the aortic override in five patients and hypoplastic right ventricle in the patient with pulmonary atresia and intact septum. Peak systolic gradients between the left ventricle and truncus (or aorta) at cardiac catheterization ranged from 20 to 47 mm Hg. Retrograde aortography confirmed a domed and stenotic semilunar valve. Cyanosis was progressive in the three patients with pulmonary atresia. Two patients had arterial anastomosis, and one had a right ventricle-pulmonary arterial graft in addition to aortic valvotomy. One of the three patients with truncus arteriosus underwent complete repair in addition to truncal valvotomy but he died in the post-operative period. The other two patients with truncus arteriosus died of intractable congestive cardiac failure before surgical intervention. It is suggested that the presence of semilunar valve stenosis in these patients adversely affects the prognosis. The myocardium is already jeopardized as a result of hypoxia in pulmonary atresia and left ventricular diastolic overload in patients with truncus arteriosus. The added burden of semilunar valve stenosis may further compromise the functional status of the myocardium.
The angiocardiographic features of 11 patients with superoinferior and criss-cross type of atrioventricular (A-V) connections are presented. These unusual ventricular relations are thought to result from postseptation disturbances of ventricular looping. The angiocardiographic appearance of criss-cross is really an illusion, and the A-V connections among these patients are either concordant, discordant or straddling. The often small right A-V valve inflow and sinus portion of the ventricle, combind with the ventricular rotational anomaly, combine to give the angiocardiographic perception of criss-cross. A review of the 11 patients from this institution and those previously reported on suggests that most patients have a transposition of malposition of the great arteries; many have a small right ventricle, and about half have pulmonary outflow tract obstruction. In addition to the obvious embryologic, morphologic and clinical implications of these distorted ventricular loops, the criss-cross A-V hearts raise questions about the various segmental nomenclatures applied to these types of congenital heart disease.
Thallium-201 myocardial imaging was performed on six patients with an anomalous left coronary artery arising from the pulmonary artery. Initial images in three children (aged 4 months, 12 months and 2 1/2 years, respectively) demonstrated anterolateral perfusion defects in agreement with the electrocardiographic localization of infarction. Repeat imaging in two patients 2 to 3 months later, after clinical improvement with anticongestive therapy, demonstrated a reduction in the perfusion defect. In 3 other patients (aged 16, 16 and 20 years, respectively) thallium-201 scans performed during exercise stress demonstrated normal myocardial perfusion 7 to 15 years after operation (ligation of anomalous left coronary in two, ligation plus bypass graft in one). Thallium-201 imaging appears to be helpful in monitoring changes in myocardial perfusion before and after medical or surgical treatment of an anomalous left coronary artery, and may shed light on the pathophysiology of the defect. A possible practical limitation of thallium-201 imaging in this condition is the difficulty of imaging subendocardial infarction.