Pulmonary Vascularity Research Articles

Congenital Heart Disease: Correlation of Pathologic Anatomy and Angiocardiography

The authors have compiled their view of the morbid and roentgenographic anatomy of congenital heart disease, presenting their extensive experience in the form of x-rays, angiocardiograms, photographs of pathological specimens, and drawings. More than 1,000 photographic reproductions of excellent quality are provided. The quality and number of these illustrations undoubtedly account for the high price of these volumes. While the authors are extravagant with expensive paper and reproductions, only brief bits of concise text, rarely filling more than one-half page, are presented. The material is divided on the basis of degrees of pulmonary arterial vascularity, and subdivided according to the presence or absence of cyanosis. As a consequence, those common defects which may be associated with pulmonary vascular obstructive disease are discussed in two different places under acyanotic and cyanotic headings. This extensive repetition scarcely seems necessary in a book devoted to pathological and roentgenographic anatomy, since the secondary effects

INFERIOR VENA CAVAL POSITION IN THE DIFFERENTIAL DIAGNOSIS OF ATRIAL AND VENTRICULAR SEPTAL DEFECTS.

The dominant features of congenital heart lesions with left-to-right shunts observed by conventional roentgen examination are increase of pulmonary vascularity and enlargement of the heart. Of particular importance is the differential diagnosis of atrial and ventricular septal defects. Increased size of the left atrium indicates a shunt distal to the atrioventricular valve, while no enlargement of the left atrium is found in atrial septal defects (2). In ventricular septal defects clinical signs of enlargement of the left ventricle are usually present. In cases of secundum atrial septal defect the left ventricle is presumably of normal size. Assessment of the size and configuration of the left ventricle on the basis of conventional roentgenography is generally considered difficult since available criteria are hard to apply in practice. In the determination of left ventricular dilatation, however, the topographical relationship of the inferior vena cava to the posterior surface of the heart in the lateral projection of the chest is a valuable diagnostic aid. It occurred to us that since left ventricular dilatation does not occur in atrial secundum septal defects but is the rule in ventricular septal defects with significant shunts, such altered relationship of the left ventricle to the inferior vena cava might be useful in differentiating these lesions. This report concerns our experience with the utilization of this sign. Methods To test the validity of this concept, all cases of ventricular and atrial secundum septal defects at the Children's Clinic of the Karolinska Hospital were reviewed. Each case was proved by cardiac catheterization and angiography. The series included some adults who were brought to the Children's Clinic for angiocardiography. All patients under four years of age were eliminated from the group because of the recognized difficulties in obtaining chest roentgenograms in full inspiration at this age. Also excluded from the series were patients in whom the degree of inspiration was so shallow as to prevent visualization of the inferior vena cava in the lateral projection. Of the patients with ventricular septal defect, only significant shunts were considered, as small shunts characteristically produce no significant alteration in cardiac contour and therefore do not constitute a problem in differential diagnosis from the atrial septal defect. It was felt that a significant shunt was present in the included cases of ventricular septal defect if cardiac enlargement and definite increase in prominence of the pulmonary vasculature were evident. Since patients with atrial septal defects typically have large shunts and radiologically detectable alterations in heart size and pulmonary vascularity, there was no necessity to eliminate any of these on the basis of size of the shunt.

TETRALOGY OF FALLOT: RADIOLOGIC EVALUATION BEFORE AND AFTER SURGICAL TREATMENT.

From 1888 (1) to 1954 tetralogy of Fallot was one of many congenital cardiac diseases which held little interest for the clinician, once the challenge of diagnosis was surmounted, and offered little hope for the patient. Rarely a child survived to adulthood or even old age, but most succumbed in their teens. Blalock and Taussig (2) in 1945 first conceived the idea of an artificial ductus in the form of a subclavian-pulmonary anastomosis. Keen interest was aroused, and other operative approaches soon followed: Potts' aortic-pulmonary anastomosis in 1948 (3), Brock's infundibular resection in 1950 (4), and Lillehei's anatomic correction utilizing a heart-lung bypass in 1956 (5). This communication is based upon a review of preoperative and postoperative postero-anterior chest films of 67 patients with tetralogy of Fallot. An attempt was made to analyze cardiac size and contour and pulmonary vascularity before and after operation. Findings were tested for statistical significance when possible. Experience wi...

CARDIAC ARREST DURING STRENUOUS EXERCISE IN A PATIENT WITH CONGENITAL HEART DISEASE

THE FOLLOWING CASE is presented in order to introduce a note of caution into consideration of the advisability of recommending unmonitored exercise for patients with heart disease. The patient is a 24 year old third year medical student who had been known to have a heart murmur since birth. He had lived a vigorously active life and had participated in high school football as well as snow skiing in more recent years. At the age of 19 he underwent a detailed cardiac work-up including right heart catheterization at another hospital. The findings at that time included a normal examination except for a grade III/VI blowing pansystolic murmur at the 4th left intercostal space. The chest x-ray disclosed a normal cardiac silhouette with normal pulmonary vascularity. The electrocardiogram disclosed right bundle branch block and left axis deviation. Cardiac catheterization disclosed systolic pressure of 30 mm. Hg. in the right ventricle and 23 in the pulmonary artery. There was O2 saturation of 78 per cent in the superior vena cava and 75 per cent in the inferior vena cava. In the high and mid right atrium O2 saturations ranged from 76 per cent to 80 per cent. Low in the atrium, just above the tricuspid valve, there was a small step-up of O2 saturation to 83 per cent and similar saturations were found in the right ventricle and pulmonary artery. The findings suggested a small shunt from left ventricle to right atrium of the type seen in the transitional form of atrio-ventricular communis. During the two weeks prior to the present event the patient and his roommate who was also a third year medical student, had undertaken to improve their physical conditioning by performing the Canadian Air Force series of calisthenic exercises.

Truncus insufficiency: Common truncus arteriosus with regurgitant truncus valve: Report of four cases

The clinical and pathologic findings in 4 cases of persistent truncus arteriosus associated with thick, nodular, incompetent semilunar valves are described. Review of the literature and theories of pathogenesis are presented. Diagnosis during life of common truncus arteriosus with truncus insufficiency is suggested by a decrescendo diastolic murmur along the left sternal border in a minimally cyanotic infant with cardiac enlargement, increased pulmonary vascularity, and a wide heart base in frontal and oblique roentgenographic views. Selective retrograde aortography should confirm the diagnosis.

The Evolution of Interventricular Septal Defect into Acyanotic Tetrad

The common congenital interventricular septal defect may evolve along one of a number of different pathways. 1. The pulmonary arteriolar bed may undergo medial and intimal changes resulting in Eisenmenger's syndrome. 2. Spontaneous functional closure of the defect may occur (2). 3. The defect may remain unchanged throughout life (maladie de Roger). 4. Infundibular pulmonic stenosis may develop through muscular hypertrophy of the right ventricular outflow tract, thereby producing an acyanotic tetralogy of Fallot. The purpose of this report is to show that transformation of a simple interventricular septal defect into acyanotic tetralogy may be clearly demonstrated on serial chest roentgenograms. We have observed this transformation in a group of 9 patients followed for periods of three to eleven years. All had one or more cardiac catheterizations and 6 had surgical confirmation of the infundibular pulmonary stenosis. Two cases will be reported in some detail. Case Reports T. G., an 11-year-old white boy, had been found to have a cardiac murmur at three weeks of age. He had been seen regularly and had periodic roentgen examinations of the chest at Grace-New Haven Community Hospital since five months of age. No cyanosis was ever noted. After the age of three he had gradual progressive diminution in exercise tolerance. The pertinent physical findings from five months to six years of age were: cardiomegaly, a precordial systolic thrill, and a Grade IV harsh systolic murmur at the third interspace at the left sternal border, with precordial transmission. The second sound at the pulmonic area was split, with normal to slightly accentuated intensity. The electrocardiogram revealed biventricular hypertrophy. Roentgen examination (Fig. 1, A and B) at five months showed cardiomegaly, increased pulmonary vascularity, left atrial enlargement, and a normal to small aorta. Between the ages of six months and ten years (at which time the patient was operated on) the heart size became normal. The second sound at the pulmonic area became single and less intense than the second sound at the aortic area. The electrocardiogram showed right axis deviation, right incomplete bundle branch block, and right ventricular hypertrophy. Roentgen examination (Fig. 1, C–D) now revealed no cardiomegaly, normal pulmonary vascularity, no left atrial enlargement, and an increase in the size of the aorta. Cardiac catheterization at the age of ten revealed a left-to-right shunt at the high ventricular level, severe right ventricular hypertension, and a gradient at the right ventricular outflow tract. The pulmonary artery pressure was normal. A right-to-left shunt was demonstrated after exercise. Several months later a 2-cm. ventricular septal defect was closed and a moderate amount of infundibular tissue was resected.

Cardiovascular findings in children with sickle cell anemia

Cardiovascular findings are described in seven children with sickle cell anemia. Clinically the following features were noted: (1) exertional dyspnea and fatigue; (2) wide fixed splitting of the second heart sound; (3) third “filling” heart sound in five of the seven cases; (4) systolic murmur of grade II intensity or louder, most prominent over the upper left sternal border; (5) roentgenographic evidence of diffuse cardiomegaly and increased pulmonary vascularity; (6) abnormal electrocardiograms in four cases. Laboratory findings of note were: (1) hemoglobins from 6.3 to 9.5 gm. per cent and hematocrit readings from 18 to 27 volumes per cent; (2) increased total blood volumes and plasma volumes in five of six patients. Right heart catheterizations in these patients revealed the following: (1) normal vena caval, right atrial, pulmonary artery and wedged pulmonary artery pressures; (2) normal or low pulmonary vascular resistances at rest and with exercise; (3) cardiac indices averaging twice normal; (4) increased stroke indices; (5) arterial oxygen unsaturation at rest and after breathing 100 per cent oxygen in all cases; (6) relatively small arteriovenous oxygen differences at rest and with exercise; (7) relatively high coronary sinus oxygen saturations; (8) an abnormal Valsalva response.

Results of surgery for ventricular septal defects∗

The surgical correction of ventricular septal defects can be accomplished today by teams experienced in extracorporeal perfusion technics with a minimum of risk and a maximum of effectiveness. There has been no operative mortality during the past two years in the series reported, regardless of the degree of pulmonary hypertension. With this degree of success it is believed that patients with significant shunt flows (40 per cent or more) should have the shunts closed without waiting for the development of enlarged hearts, increased pulmonary vascularity and pulmonary hypertension. Operative intervention is delayed in infants less than one year of age or less than 20 pounds in weight because of the increased risk.

The radiographic evaluation of pulmonary vasculature in children with congenital cardiovascular disease.

The availability of surgical procedures for the treatment of congenital malformations of the heart in children has made necessary an increasing accuracy in their diagnosis. Many malformations require, for accurate identification, an analysis of data obtained clinically, by conventional chest radiography, and by cardiac catheterization and∕or cardioangiography. Since the conventional chest film is usually the most common study in conjunction with clinical examination, it is important to use it as fully and accurately as the method allows. The status of the pulmonary vasculature is one criterion in reaching a radiologic diagnosis by the relatively simple technic of teleroentgenography. A radiologic diagnosis of increased or decreased pulmonary vascularity may require actual measurements of the vessels on the chest film, but is usually obtained from a general impression of the shadows of the pulmonary fields. Measurements have been undertaken in adults (5), but in children with congenital heart disease, this...

Method of radiologic diagnosis of congenital heart disease in children.

The systematic application of roentgenologic criteria here proposed permits the objective classification of patients with congenital heart disease. The basic criterion is the degree of pulmonary arterial vascularity, which may be increased, normal, or decreased. The first class, marked by increased pulmonary vascularity, is the largest, it includes interventricular and interatrial septal defects, the most frequent congenital heart lesions. The second largest class, marked by decreased pulmonary vascularity, includes another frequently seen lesion, the tetralogy of Fallot. The third class, with normal pulmonary vascularity, includes such lesions as coarctation of the aorta. Conventional films and fluoroscopy may need to be supplemented by retrograde aortography and angiocardiography; in addition, roentgenographic findings during heart catheterization often give valuable diagnostic clues. Analysis of the roentgenologic features of a case, obtained by conventional x-ray examination without contrast, considered along with the simple clinical data, will generally yield a working diagnosis.