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Perloff Clinical Recognition Of Congenital Heart Disease Pdf 28

Out of 18 patients, 12 (66.6%) had previously undergone palliative procedures before corrective surgery. Of these patients, two (11%) had a central shunt, two (11%) had patent ductus arteriosus (PDA) stenting, one (2.9%) had right ventricular outflow tract (RVOT) stenting, three (16%) had pulmonary banding, two (11%) had an aortic arch repair and pulmonary banding, and two (11%) had a bidirectional Glenn (BDG) shunt. After 3D cardiac modeling, univentricular heart palliation was performed in six patients (33%; three BDG, three Fontan procedure) and biventricular repair was conducted in 12 patients (67%). All cases survived with good clinical outcomes and no major complications.

perloff clinical recognition of congenital heart disease pdf 28

The natural history of VSDs is largely dependent on the size and location of the defect and the downstream pulmonary vascular resistance [4]. Thus, patients with large VSDs in adult life present with congestive heart failure or pulmonary hypertension and right heart failure; thus, closure would be undergone in early life, while adults with small defects are usually asymptomatic. In addition, it is not easy to obtain accurate data of relatively asymptomatic congenital heart disease after patients become adults. One of the major reasons are the following doctor changes from a pediatric cardiologist to an adult cardiologist. So, evidence in the medical literature concerning elderly patients with VSD is lacking, particularly for those over 80 years old (5, 6, 7); one of the three was a finding on autopsy [5].

However, for elderly VSD patients, the surgical indication is less clear. A conservative follow-up is often recommended unless a significant heart failure is revealed, with prophylaxis against infective endocarditis [6]. But, one must recognize that with a progressive increase in systemic vascular resistance with age, the degree of shunt can increase, making VSDs become more hemodynamically significant later in life [4]. So, some surgeons recommend VSD closure as long as pulmonary vascular obstructive disease is not prohibitive [4].

In this case, off-pump CABG, which means leaving VSD and/or valvular disease, was another possible surgical option to avoid too extensive treatment. However, despite its asymptomatic history, high-flow VSD, in addition to MR and TR, would likely lead to perioperative and future heart failure. Fortunately, his physical condition was considered able to withstand a complex operation; thus, we decided to perform CABG, MAP, TAP, and VSD repair simultaneously. His postoperative course was uneventful.

incidence of 4% in clinical series and 12% in autopsy studies. The hearts in which the opening is proximal to the displaced tricuspid valve, a left ventricular to right atrial shunt may occur as shown in Figure 20.

Whereas congenital heart disease was mainly an anatomical curiosity, rheumatic fever was rampant all over the world, but little attention was given to the cardiac findings. Thomas Sydenham made the first clinical description of rheumatic fever in 1676 (9) and later described chorea. Vieussens in 1715 (10) described an autopsy of rheumatic heart disease showing mitral stenosis and aortic regurgitation (11). In 1835, Bouillard of France recognized that inflammation of the pericardium and heart valves was most frequently due to rheumatic fever. Cheadle in 1889 (12), in his Harvarian lecture, described child after child with acute rheumatic fever and serious cardiac disease. He often commented how one or the other parent was disabled as a result of rheumatic heart disease and was one of the first to recognize that acute rheumatic fever in childhood led to later disabling rheumatic heart disease.

In the early 20th century, almost every city had a convalescent home for children with rheumatic fever. Children with rheumatic fever were treated and followed by general pediatricians. Specialty clinics were developed, and children with heart murmurs tended to be followed in a rheumatic fever clinic. Pediatricians who had an interest in cardiology but little training saw children with rheumatic heart disease and innocent murmurs and some with congenital heart disease. It was in such a clinic that Helen Taussig (13), often called the mother of pediatric cardiology, began her career.

Although it had been recognized that pharyngitis often preceded acute rheumatic fever, it was not until the middle of the 20th century that the crucial role of β hemolytic Streptococcus in the pathogenesis of rheumatic fever was confirmed. Overcrowding and poor social conditions allowed streptococcal infections to flourish. Improvement in social conditions brought about a decrease in the incidence of rheumatic fever. However, the discovery of sulfonamide and penicillin allowed streptococcal infections to be treated effectively, and with this prophylaxis, recurrent episodes of rheumatic fever could be prevented. With the triumph over rheumatic fever, congenital heart disease became the predominant form of heart disease in children.

In 1930, Edwards A. Park placed Helen Taussig in charge of the cardiac clinic at the Harriet Lane Home and had a fluoroscope installed. He had been impressed with May Wilson's ability to use the fluoroscope to study heart size in children with rheumatic heart disease. He encouraged Helen Taussig to learn to use the fluoroscope so that she might learn more about congenital malformations of the heart. Late in the 19th century, Roentgen (14) had developed x-ray, and in the same year, Williams (15) described the use of the fluoroscope. Einthoven in 1902 (16) developed the ECG. By the time Taussig began her clinic, these tools were available. Using the ECG and fluoroscopy, Taussig was able to correlate physical findings with the pathology noted at autopsy. She attributed her knowledge of pathology to what she learned from Maude Abbott. The Canadian pathologist Maude Abbott became an assistant curator at the medical museum at the McGill, which William Osler had started. When Osler met Abbott in 1898, he encouraged her to become interested in congenital heart disease because he thought that the teaching possibilities of such a museum were great. In 1907 (17), she published her atlas, which was to become very important as advances followed in cardiac surgery and pediatric cardiology.

On August 26, 1938, Robert Gross, at the age of 33, successfully ligated the patent ductus of a 7-y-old girl at the Children's Hospital of Boston (18). Congenital heart disease was suddenly not just an interesting oddity but also a treatable condition. It had been recognized that infants with tetralogy of Fallot were often pink until the ductus arteriosus closed. Taussig reasoned that if a ductus could be ligated, then why not construct a ductus. She tried to encourage Gross to consider such an operation, but when he declined, she found Alfred Blalock a willing collaborator. Blalock had already performed anastomoses between the left subclavian artery and pulmonary artery in his attempt to learn more about pulmonary artery hypertension. In November 1944 (19), Blalock anastomosed the left subclavian artery to the pulmonary artery and changed the life of a severely cyanotic child with tetralogy of Fallot. The Blalock-Taussig operation, as it was called, soon had worldwide recognition. The following year, Crayfoord and Nylin (20) from Stockholm, Sweden, successfully repaired a coarctation of the aorta with an end-to-end anastomosis. Sir Russell Brock in 1948 (21) successfully performed relief of pulmonary stenosis by a transventricular approach. All of these surgical procedures were performed on the beating heart. To repair intracardiac lesions, cardiopulmonary bypass was needed. In 1955 (22), Walt Lillehei and associates in Minneapolis reported the results in 32 patients with ventricular septal defect, tetralogy of Fallot, and atrioventricular communis defects using a human cross-circulation technique. Fortunately, an effective, mechanical form of cardiopulmonary bypass soon replaced this daring surgery. John H. Gibbon, Jr. (23), of Philadelphia is considered the architect of extracorporeal circulatory support, but success awaited modification of his original design. In 1955, John Kirklin (24), at the Mayo Clinic, reported eight cases that were treated successfully with the use of mechanical cardiopulmonary bypass.

These remarkable advances in the surgical treatment of congenital cardiac defects were accompanied by similar remarkable advances in accurate diagnoses and understanding of the pathophysiology of cardiac defects. Forssman in 1929 (25) passed a catheter into his own heart, but it was not until 1932 that Richardson and Cournand began performing cardiac catheterizations in humans. With the advent of surgery for cardiac defects, it became essential that correct diagnoses be made preoperatively. In 1949, Cournand (26) and his associates in New York, working with Janet Baldwin, a pediatric cardiologist, reported on cardiac catheterizations in congenital heart disease. At the same time, Bing et al. (27) were studying children with cardiac defects at Johns Hopkins. Catheterization laboratories developed in many centers, including the Mayo Clinic under Earl Wood. Even before pediatric cardiac catheterization was available, Costellanos (28) in Cuba reported his experience with angiograms in children in 1939. In the United States, angiography was not used widely until the late 1940s and early 1950s. By the late 1950s, pediatric cardiology centers had developed all over the country, and by that time, cardiac catheterization and angiography were usually present in a single laboratory.

Early enthusiasm for open-heart surgery was somewhat dampened by the high mortality of children after apparent successful repair of a large ventricular septal defect. Death came in the early postoperative period after apparent successful surgical closure. Understanding of the pulmonary vascular bed was limited. Donald Heath and Jesse Edwards (29) studied the pathology of the pulmonary vascular beds in patients with left-to-right shunts and developed a grading system describing the severity of medial hypertrophy and intimal change in the arterioles. Physiologic studies increased knowledge about pulmonary hypertension and the concept of pulmonary vascular obstructive disease as a complication of pulmonary artery hypertension became recognized. Unfortunately, many of the children with large ventricular septal defects and pulmonary hypertension had developed pulmonary vascular disease, and when they underwent surgical closure, the results were disastrous.


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