Whither paediatric cardiology?
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Biomedical subjects
Publications and source records attributed to J Somerville.
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Cardiologists assuming responsibility for adults with congenital heart disease must have knowledge of electrophysiologic, valvular (native valves), prosthetic (valves, patches and conduits), ventricular (especially chamber function), vascular (especially elevated pulmonary vascular resistance) and noncardiovascular residua and sequelae. Acquired cardiac and noncardiac diseases coexist in older adults with postoperative congenital heart disease and add to the physician's responsibilities.
Sixteen patients presenting on 21 occasions with atrial flutter in association with complex congenital heart disease were treated by intracardiac stimulation techniques combined with activation mapping. Nineteen episodes of atrial flutter were successfully converted to sinus rhythm. In the remaining two episodes atrial fibrillation was induced with spontaneous conversion to sinus rhythm within 12 hours in one episode and immediate DC cardioversion to sinus rhythm in the other. Intracardiac stimulation techniques were highly successful in this group and allowed reliable conversion to sinus rhythm without general anaesthesia and high energy cardioversion. In patients with atrial flutter associated with congenital heart disease intracardiac stimulation techniques should be tried first.
A 35 year old woman with multiple pulmonary arterial stenoses and occlusions was admitted with increasingly severe breathlessness. Balloon angioplasty of a life threatening stenosis in a remaining pulmonary artery branch was attempted on two occasions but without success. When a self-expanding stainless steel stent was inserted at the site of stenosis after a further angioplasty acute localised pulmonary oedema developed as blood flow distal to the lesion increased considerably. This was followed by a slow and dramatic improvement in the patient's condition. She no longer required an urgent transplant and resumed an active life.
The data available on 111 patients with congenitally corrected transposition and 2 adequate ventricles managed over the 20-year period to 1988 were reviewed retrospectively. The ages of survivors ranged from 1 to 58 years (median 20) and all but 10 had additional anatomic abnormalities. Tricuspid valve abnormalities were more prevalent in patients symptomatic with heart failure (26 of 43 patients) than those whose main problem was cyanosis (11 of 52 patients); all dysplastic or Ebstein valves were at least moderately incompetent. Intracardiac repair of the lesion was attempted in 51 patients with 11 early deaths; in multivariate models, the risk factors for early death or a bad early outcome or poor result 6 months later related to poor preoperative symptomatic status (especially from heart failure), impaired right ventricular function, heart block and younger age at surgery. Patients with more than mild preoperative tricuspid regurgitation whose valves were not replaced did very poorly. Thus, patients symptomatic from heart failure should probably be repaired early in the natural history of the disease, before the systemic right ventricles dilate. By contrast, the course of patients who were predominantly cyanosed was more stable in early childhood and their surgical outcome was less compromised by poor preoperative symptomatic status; their intracardiac repair can probably be delayed until symptoms become unacceptable.
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Aortic atresia is a rare congenital cardiac defect. It usually leads to death in the neonatal period. A patient with aortic atresia has survived to the age of 24 years without any surgical procedure. In view of the uncertain results of reconstructive surgery this case may have therapeutic implications.
The anatomic incidence of left ventricular false tendons has been determined in 100 hearts obtained from consecutive autopsies on patients who had a wide variety of cardiac conditions. In this series, an anomalous band was found in 34 cases. A retrospective two-dimensional (2D) echocardiographic study was able to identify false tendons in only 18 percent of cases with anatomic evidence of them, indicating 2 mm as the limit of resolution of 2D echocardiography even with appropriate projections. Finally, the histologic examination has shown false tendons to contain conduction tissue and thus it is assumed to be intracavitary radiations of the bundle of His. This last consideration introduces new physical and electrophysiologic implications.
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'Grown-up' congenital cardiac patients are an increasing medical community, not at present being properly cared for in any country. Information on over 2000 patients aged 15-65 years with congenital heart disease who have been treated in the National Heart Hospital provides the data for this report.
This study establishes normal values and growth curves of the aortic root to determine when it is unusually small. The aortic root diameter was measured by 2-dimensional echocardiography in 188 normal subjects (group 1) and in 33 patients (group 2) with left ventricular outflow obstruction in whom the diagnosis of small aortic root was made during surgery for aortic root and valve replacement. In group 2 the aortic root was also measured by M-mode echocardiography and was compared to measurements during surgery. Growth curves of the normal aortic root were obtained. In group 2 the aortic root was smaller than normal except in 3 patients. When corrected for body surface area all were smaller than normal. The 2-dimensional echocardiographic and surgical measurements were almost identical. M-mode values were higher (p less than 0.01). The normal values and growth curves of the aortic root diameter are thus established and the small aortic root can be identified preoperatively.
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Thirty-six patients were studied with magnetic resonance (MR) imaging 2-29 years after repair of coarctation of the aorta, and the findings were compared with clinical data and results of echocardiography, catheterization, angiography, and surgery. A satisfactory echocardiographic examination of the isthmus was achieved in 28 patients. Comparison with data from gradient measurements at catheterization and Doppler ultrasound shows that the reduction of lumen diameter correlates well with the gradient and that restenosis at the site of the repair can be suspected when the percentage of stenosis at the isthmus is greater than 50%. MR imaging showed a Dacron patch aneurysm in three patients. Cine MR imaging showed systolic signal loss in the ascending aorta that was presumed to be due, among other factors, to turbulent flow generated from a bicuspid valve. Similar signal loss was seen in the descending aorta distal to the site of the coarctation repair, but this was not related to the presence or absence of a gradient. Aortic compliance measured from transverse MR systolic and diastolic images was abnormally low, suggesting it may be a factor causing systolic hypertension after coarctation repair. MR imaging is a reliable and accurate noninvasive method likely to supersede other methods of assessing the aorta and isthmus following coarctation repair.
The Mustard operation in infancy and childhood has successfully palliated many patients with transposition of the great arteries who have now survived to adulthood. Right ventricular dysfunction and tricuspid regurgitation are important determinants of late morbidity and mortality. The value of noninvasive magnetic resonance imaging (MRI) in the assessment of cardiac function and anatomy 9 to 20 years after this procedure has been investigated, and compared with findings on echocardiography, radionuclide ventriculography and angiography in 17 adult patients. Ejection fractions measured by MRI were higher compared with radionuclide ventriculography. The correlation for the left ventricle was closer (r = 0.75) than for the right ventricle (r = 0.49). Tricuspid regurgitation was assessed by Doppler echocardiography and by MRI using the right/left ventricular stroke volume ratio. The mean stroke volume ratio in those with Doppler evidence of tricuspid regurgitation was 1.6:1 compared to 1.1:1 in those without, and this difference reached significance (p less than 0.01). The anatomy of the great arteries was clearly visible in all patients. Five patients had a residual ventricular septal defect which, with the exception of 1 small defect, was easily visualized. The intraatrial baffle was best seen in transverse slices, and the systemic venous connection showed as a relatively narrow channel lying in the posterior part of the cavity. In general, baffle anatomy was easier to assess on 2-dimensional echocardiography.(ABSTRACT TRUNCATED AT 250 WORDS)
The effect of experimental balloon dilation on seven excised specimens of classic coarctation was assessed histologically. Widening of the lumen was achieved in all specimens by tears in the vessel wall. The tears extended through the intima in one specimen and through both intimal and medial layers in six specimens. In view of the extent of wall damage, we postulate that there would be serious long-term complications following balloon dilation for the treatment of native coarctation.
An anomalous origin of the left anterior descending coronary artery from the pulmonary trunk with the right and left circumflex arteries arising from the aorta is very rare and the diagnosis made only at necropsy. An anomalous coronary artery was suspected owing to unexplained cardiomegaly in a 17 month old girl and the anatomy was defined by angiography. The anomalous vessel was successfully reimplanted into the aorta.
The clinical presentation, diagnosis, and surgical treatment of 63 patients with doubly committed subarterial ventricular septal defects (DCVSD) were analyzed retrospectively. The patients were divided into three groups. Thirty-one patients had severe congestive heart failure in infancy and presently have a large ventricular septal defect that has no tendency to close or to produce aortic valve regurgitation (Group 1). Ideally, these defects should be closed in infancy, and the transpulmonary approach is recommended to achieve closure. In the first group, there was one death in a patient with a hypoplastic right ventricle. In Group 2, nineteen patients had aortic valve prolapse or aortic valve regurgitation. The DCVSD were moderately large or small. A number of DCVSD in Group 2 patients had maintained the normal offsetting of the arterial valves. These defects must be closed by the time mild aortic valve regurgitation has occurred. In Group 2, there was one late death in a patient who developed subacute bacterial endocarditis. The two patients who had severe aortic valve regurgitation required aortic valve replacement and underwent multiple surgical procedures to replace calcified bioprostheses. Group 3 comprised 13 patients who were diagnosed with tetralogy of Fallot. All of these patients had a large DCVSD with aortic valve overriding. All Group 3 patients survived radical repair of the defect, which required a transannular patch in most cases (76%).
A newborn with preductal coarctation and ventricular septal defect had a subaortic aneurysm which protruded into the left atrium causing intractable pulmonary oedema. The subaortic aneurysm was congenital. It was clearly identified by cross-sectional echocardiography which, in serial studies, showed it to be increasing in size. Predisposing to its development was the wide area of aortic and mitral discontinuity in association with the concordant ventricular connexion of the great arteries. The child remained in worsening pulmonary oedema after relief of the coarctation and banding of the pulmonary trunk and died after attempted relief of the left atrial obstruction.