Radiological case of the month. Tetralogy of Fallot with absent pulmonary valve.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to T W Riggs.
Explore the source record for details and available documents.
Echocardiographic findings and cardiac catheterization data were evaluated in 18 infants less than 1 year old in order to define anatomical or pathophysiological features that were associated with early cardiac decompensation. The infants could be divided into three groups: Group I (10 patients) had left ventricular dilatation and depressed contractility in response to the severe systemic hypertension. Group II (3 patients) had marked myocardial hypertrophy In response to the systemic hypertension. Group III (5 patients) were the youngest patients and had findings of right ventricular volume overload and pulmonary hypertension. This study demonstrates that, in early infancy, the ventricular response to simple coarctation of the aorta is variable in infants in a state of cardiac decompensation. The different echocardiographic and hemodynamic findings may be a consequence of the lesion exerting its influence at various stages of the patients' intrauterine or postnatal life. In most patients, resection of the coarctation results in rapid normalization of the echocardiographic findings.
Explore the source record for details and available documents.
Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic "goose-neck" deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.
Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.
Two-dimensional echocardiograms and angiocardiograms were obtained in six infants and children, each with complete transposition of the great arteries, a ventricular septal defect and subvalvular pulmonary stenosis. In each case, the subpulmonary stenosis resulted from redundant tricuspid valve tissue which protruded through the ventricular septal defect and into the left ventricular outflow tract. Angio-echocardiographic correlations demonstrated features that allowed preoperative recognition of this unusual type of subpulmonary stenosis. The left ventricular angiogram in an anteroposterior orientation revealed a characteristic filling defect in the outflow tract during systole in each patient. Echocardiographic images of the left ventricular outflow tract and of the tricuspid valve demonstrated subpulmonary stenosis caused by protruding redundant tricuspid valve tissue (resembling a "pouch") in all six patients. These findings were contrasted with those from three patients with normally related great arteries and a similar, but nonobstructive, tricuspid valve pouch. Echocardiographic and angiocardiographic examination and correlation allowed preoperative diagnosis of this unusual form of subpulmonary stenosis in complete transposition of the great arteries. This is especially important because limited left ventricular intraoperative exploration may fail to reveal subpulmonary obstruction as the pouch may be flaccid in a relaxed arrested heart.
Two-dimensional echocardiograms of the mitral valve orifice area were obtained in 50 normal pediatric subjects, 15 patients with congenital mitral stenosis and 7 patients with tricuspid atresia. The mitral area was measured near the tips of the mitral valve leaflets from a diastolic cross-sectional image of the left ventricle. The cardiac images were recorded on videotape and later transferred to video disc for high resolution contour tracing. Contour analysis was performed by a special purpose microcomputer system for calculation of the enclosed calibrated area. In normal patients, there was an excellent correlation (r = 0.95) between mitral valve area (MVA) (in cm2) and body surface area (BSA) (in m2) described by MVA = 4.83 X BSA - 0.07. Each patient with mitral stenosis had a mitral valve area that measured less than the third percentile predicted from the normal regression formula. In eight patients in whom the Gorlin formula could be applied, there was excellent correlation (r = 0.95) between echocardiographic and hemodynamic measurements of mitral valve area. Each patient with tricuspid atresia had a very large mitral valve area (greater than 99th percentile of normal). It is concluded that noninvasive measurement of mitral valve orifice area can be accurately achieved by two-dimensional echocardiography in pediatric patients with congenital mitral stenosis, allowing serial measurement of their mitral valve area.
Explore the source record for details and available documents.
Two-dimensional echocardiographic examinations were performed in seven infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCA), in one infant with myocardial infarction and left main coronary obstruction, and in eight with critical valvar aortic stenosis (AS). Comparative qualitative echo density assessment demonstrated that each infant had a marked increase in the echo density of one or both left ventricular papillary muscles. The increased echo density was considered to represent fibrosis and scar formation as a result of ischemia and infarction. Pathologic proof of excessive fibrosis of the papillary muscles was obtained in three cases. In an additional case, calcification of the papillary muscle was noted on fluoroscopic examination. The echocardiographic appearance of papillary muscle fibrosis provides a useful indicator of severe subendocardial ischemia in patients with either critical AS or ALCA.
Twenty-seven patients (3 to 22 years) underwent the Fontan operation. Seventeen had tricuspid atresia and 10 had other complex lesions. Sixteen patients had a Glenn shunt (12 prior to, three simultaneous with, and one following the Fontan operation). One early death (3.7%) occurred in a 4 1/2-year-old child with tricuspid atresia II-C, previous pulmonary artery banding, and a closing ventricular septal defect (80 mm Hg gradient). There were two late deaths (7.4%) from Candida sepsis, after 4 and 6 months, respectively. The 24 patients who survived the Fontan operation had postoperative hospital stays of 6 to 90 days (average 18). Patients with tricuspid atresia and an established Glenn shunt (nine patients, Group I) had postoperative hospital stays of 7 to 19 days (average 9.5), and none had significant pleural or pericardial effusions. Patients with tricuspid atresia without a Glenn shunt (seven patients, Group II) had postoperative hospital stays of 6 to 60 days (average 17.5), with three having significant effusions. Of the patients with other complex lesions, all without an established Glenn shunt, five had significant effusions. Four additional major complications (two tricuspid patch disruptions with ineffective pulmonary blood flow and two complete occlusions of a valved conduit) were encountered in which the Glenn shunt proved lifesaving. We believe that an established Glenn shunt played a major role in attaining minimal postoperative hemodynamic instability, effusions, renal failure, and mortality in our patients. The Glenn shunt should be considered in patients who are less than ideal candidates for the Fontan operation.
A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.
Explore the source record for details and available documents.
A previously healthy 8-year-old girl with a normal heart had development of tricuspid valve staphylococcal endocarditis. Serial two-dimensional echocardiograms first demonstrated an enlarging vegetation and later showed reduction in its size coincident with a pulmonary embolus. The occurrence of the pulmonary embolus along with persistent fevers, despite adequate antibiotic treatment, prompted surgical intervention. Surgical excision of a large residual vegetation, valve debridement, and pulmonary embolectomy led to prompt subsidence of fever and improvement in the patient's condition. Three months after surgery, the child remained asymptomatic with minimal tricuspid insufficiency. However, by eight months after surgery, the hemodynamic consequences of the tricuspid insufficiency had progressed, and the patient is mildly symptomatic. Additional tricuspid valve repair or replacement is expected.
Two-dimensional echocardiographic prospective diagnosis of truncus arteriosus was made in 7 infants. Two infants had truncus arteriosus type I, 3 patients had truncus arteriosus type II, 1 infant had truncal valve stenosis with an interrupted aortic arch, and 1 had type IV truncus arteriosus with pulmonary hypertension. Multiple imaging views were utilized to confirm the diagnosis. The parasternal long-axis view demonstrated the great vessel-ventricular septal override and the origin of the pulmonary arteries from the posterior aspect of the ascending truncus. The suprasternal notch image facilitated identification of the left- or right-side aortic arch and the origin of the pulmonary arteries from the truncus. Transverse imaging sections at the base of the heart facilitated identification of the pulmonary artery origin of truncus arteriosus type I. Subcostal coronal and sagittal views imaged the common truncus and the ventricular septal defect. These echocardiographic images were contrasted with and discriminated from those of an infant with aorticopulmonary window with intact ventricular septum. Although cardiac catheterization and angiography may be required to assess pulmonary arterial pressure, pulmonary vascular resistance, and the distal pulmonary arterial anatomy in truncus arteriosus, 2-dimensional echocardiography can be used to correctly establish the morphologic diagnosis of truncus arteriosus in infants.
The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized froma an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.
Explore the source record for details and available documents.
A 6-month-old infant with anomalous left coronary artery underwent a direct left subclavian-left coronary artery anastomosis without the use of cardiopulmonary bypass. Subsequently, mitral valve replacement was performed for severe mitral insufficiency diagnosed echocardiographically to be due to isolated papillary muscle infarction. To our knowledge, this is the youngest patient to have undergone both procedures during the first year of life with excellent results on follow-up studies. The case demonstrates the advantages of using direct subclavian-coronary artery anastomosis and 2-dimensional echocardiography in the management of patients with anomalous left coronary artery.
Quadricuspid pulmonic valve is a rare congenital anomaly which appears to occur in the absence of other cardiac or systemic anomalies. It predominates in males and tends to be clinically quiescent. The first case of quadricuspid pulmonic valve in a live newborn infant diagnosed by two-dimensional echocardiography is presented here with a review of the literature.