Surgical correction of ventricular septal defects in black and white children--an 11-year study.

The clinical and ECG features, anatomical subtypes and outcome in 309 children (169 black; 140 white; 58% female) who underwent surgical closure of ventricular septal defects (VSDs) are presented. Clinical presentation was more severe in the black children; with recurrent pulmonary infections in 65% blacks compared with 34% whites. Congestive cardiac failure was noted in 60% blacks and in 37% whites. At cardiac catheterisation a left to right shunt greater than 2.5/1 was found in 45.5% of the black and in 39.3% of the white children. Severe pulmonary hypertension (greater than 80% of systemic systolic pressure) was evenly distributed in both groups. Of the 140 white children, 74.3% underwent surgery under the age of 2 years compared with 68.6% of the 169 blacks. A perimembranous VSD was found in 65% of patients in each group. Infundibular (muscular outlet) defects were observed in 28.6% of white and 30.8% of black children. Left axis deviation (LAD) on ECG was found in 11.9% of white and 9.2% of black patients; and 93% of the total of 27 cases had a perimembranous defect. Early mortality was 3.6% in white and 7.1% among the black children. Of the 17 fatal cases in the total group, 16 had severe pulmonary hypertension. It is concluded that: (i) operative results compared favourably with those reported elsewhere; (ii) the anatomical subtypes occurred with equal frequency in both ethnic groups; and (iii) this was also the case for LAD on ECG, which was most commonly associated with a perimembranous VSD.

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.

Aortic arch interruption in the neonate, with emphasis on early diagnosis and management.

Interruption of the aortic arch (IAA) is a rare but lethal congenital cardiovascular anomaly. Over a 3-month period we encountered 5 neonates with IAA, all of whom presented in the 1st week of life with congestive cardiac failure and diminished or absent pulses. All 5 underwent surgery, and 3 died from causes unrelated to the surgical repair. The diagnosis of this condition is difficult to make, although the absence of pulses may provide an important clinical clue (particularly if the left brachial and femoral pulses are involved). Using prostaglandins to re-establish ductal patency may be life-saving in these patients, since this restores blood flow to the lower trunk and kidneys. Administration of oral prostaglandin E2 to infants in whom this anomaly is suspected before they are referred to a tertiary care centre is therefore vital.

Is the shunt patent?

A novel technique for auscultating an aortopulmonary shunt murmur utilizing the principle of conduction of sounds through a closed air system is described.

Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases.

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.

Valvuloplasty for rheumatic mitral valve disease. A surgical challenge.

From January 1981 through February 1985, 241 patients with rheumatic mitral valve disease (mean age 21.5 +/- 11.8 years) were subjected to comprehensive mitral valvuloplasty. One hundred seven patients (44.4%) were 15 years or younger and 63 (26.1%) were 12 years or younger. One hundred seventy five patients had pure or predominant regurgitation (mean age 19.3 +/- 10.7 years) and 40 (16.6%) had active rheumatic carditis at the time of the operation. Almost all patients (229) were in New York Heart Association Functional Class III or IV. The techniques used included shortening of anterior leaflet chordae tendineae (136 patients), resection of secondary, tertiary, and basal posterior leaflet chordae (156 patients), commissurotomy (113 patients), and implantation of a Carpentier ring (164 patients). Current operative mortality is 1.9%. The survivors were followed up for 576 patient-years (mean 2.64 +/- 1.32 years). Late mortality was 2.60% per patient-year and was valve related in 1.04% per patient-year. Reoperation was required in 25 patients (4.34% per patient-year), mostly (72%) in the first year. There were only two cases (0.35% per patient-year) of thromboembolism and three cases (0.52% per patient-year) of infective endocarditis. Hence valve failure occurred at a linearized rate of 6.08% per patient-year but was fatal in only 22% of the patients. There was no relationship between valve failure and the type of lesion or procedure performed, but reoperation was required more frequently in patients aged 12 years or less (7.33% per patient-year) than in those older than 12 years (3.29% per patient-year) (p less than 0.05). Actuarial survival rate at 41/2 years was 90%, and 82% of the patients were free from valve-related complications. Valve function after valvuloplasty was assessed clinically. Eighty-four percent of the patients had a good immediate result, but this figure dropped to 69% at the end of the follow-up period (p less than 0.05). The remainder had moderate valve dysfunction. However, 85% of the patients remain in New York Heart Association Functional Class I. Mitral valvuloplasty is an excellent alternative to valve replacement in young patients with rheumatic mitral valve disease. Persistent or reactivated rheumatic carditis may be a significant factor of valve failure, and penicillin prophylaxis is mandatory after operation.

St. Jude Medical valve replacement. An evaluation of valve performance.

St. Jude Medical cardiac valve replacement was performed in 791 patients: 335 had aortic, 330 mitral, and 126 had multiple valve replacements. Follow-up extended from 4 to 64 months (mean 34 months) with a cumulative postoperative survival of 2,111 patient-years. The overall actuarial survival rate at 5 years was 76% +/- 3%. Late valve-related mortality occurred in 28 patients (1.33%/pt-yr) and was most often caused by anticoagulant-related hemorrhage. The linearized incidences of valve failure, thromboembolism, thrombotic obstruction, prosthetic valve endocarditis, periprosthetic leak, and all valve-related complications were as follows: 1.80% /pt-yr, 2.45% /pt-yr, 0.52% /pt-yr, 0.33% /pt-yr, 0.14% /pt-yr, and 4.0% /pt-yr, respectively. Actuarially, 94% +/- 2% of patients were free of valve-related mortality at 5 years; the corresponding figures for valve failure, thromboembolism, thrombotic obstruction, and all valve-related complications were 91% +/- 2%, 89% +/- 2%, 96% +/- 1%, and 83% +/- 3%, respectively. Prosthetic valve endocarditis was uniformly fatal, and 45% of patients with thrombotic obstruction died. The greater incidence of thrombotic obstruction after mitral valve replacement was statistically significant. The performance of the St. Jude Medical valve compares most favorably with other substitute valves. Nevertheless, it retains all the imperfections and hazards of other mechanical valves, most notably, thromboembolism and thrombotic obstruction.

Traumatic rupture of the thoracic aorta. A report of 14 cases and a review of the literature.

The major controversy in the surgical repair of acute traumatic rupture of the aorta (TRA) is about the need for distal perfusion and whether or not the benefits of clamping justify the risks involved. To resolve this issue, our experience and 596 cases of acute TRA reported in the English-language literature were reviewed. The operative mortality and incidence of paraplegia among patients who underwent total or partial cardiopulmonary bypass, local temporary aorto-aortic shunting or simple cross-clamping were analysed statistically. The respective mortality rates after the above three procedures were 16,7%, 11,4% and 5,8%, and mortality was significantly increased when distal perfusion techniques were used (P less than 0,01). The incidences of paraplegia among patients who underwent partial left heart bypass, temporary shunting and simple aortic cross-clamping were 2,2%, 2,3% and 5,8% respectively (the differences did not reach statistical significance). Methods of management are discussed. Simple aortic cross-clamping remains the method of choice in uncomplicated cases of acute TRA.

Mitral valve replacement in the elderly.

The results of elective mitral valve replacement between January 1978 and June 1982 in 35 patients aged over 70 are reported. The early mortality was 22,8% and late mortality 17,1%, after a mean follow-up of 25,1 months. Patient-related risk factors included chronic mitral stenosis, often after a previous closed mitral valvotomy, increased cardiothoracic ratio, pulmonary arterial hypertension, functional tricuspid incompetence and a raised left ventricular end-diastolic pressure.

Surgical treatment of aneurysms of the ascending aorta associated with severe aortic regurgitation.

From January 1979 to June 1982 31 patients have had simultaneous ascending aortic aneurysm repair and aortic valve replacement. Fifteen patients (group 1) received a composite graft; seven patients (group 2) had separate aortic valve and supracoronary ascending aorta prostheses; and nine patients (group 3) had aortic valve replacement and "tailoring" of the ascending aorta. The mean age was 50 (SD 14) years. Nine patients had acute dissection, five with the coronary ostia affected. Emergency surgery was performed in 10 cases. There were six early deaths (19.4%), none of them due to technical complications during surgery. The mortality rate was 56% for patients with acute dissection operated on as an emergency and 4.5% for patients having elective operations. Appreciable haemorrhage occurred in four patients (12.9%). No neurological complications occurred. There was one late death. The survivors were followed up for one to four years. There was one case of recurrence of aneurysm. No ischaemic complications resulted from coronary reimplantation. There were no significant differences in the results of the three groups. Simultaneous ascending aortic aneurysm repair and aortic valve replacement can be accomplished with an acceptable mortality rate and little morbidity.

Hypothermia and circulatory arrest for surgical resection of aortic arch aneurysms.

In the 6 year period 1976 through 1981, 13 patients had surgical correction of aneurysms of the aortic arch with the use of deep systemic hypothermia (15 degrees to 24 degrees C) and partial (lower body only) or complete circulatory arrest. Three pathological groups were recognized: Group I (seven patients), with involvement of the aortic arch only; Group II (two patients), with extension of disease from the arch into its major vessels; and Group III (four patients), with predominant involvement of the major vessels. In the first eight patients (1976 to 1979), the carotid arteries were perfused directly with circulatory arrest of the rest of the body. Three of the eight patients (37.5%) died, two of cerebral complications and one of respiratory failure. Another patient had a nonfatal neurologic complication. In the last five patients (1980 to 1981), the carotid arteries were not perfused and variable periods of cerebral ischemia under hypothermic protection (18 degrees C) were permitted. All patients survived, and only one showed transient, minor neurologic changes. Our current recommended technique includes deep systemic hypothermia (15 degrees to 18 degrees C) using femoro-femoral bypass, complete circulatory arrest, and temporary occlusion of the carotid arteries. Additional protection of the myocardium is achieved by cold potassium (20 mEq/L) cardioplegia. Repair of the aneurysm is performed from within the aortic arch in a bloodless field. The hitherto high mortality and morbidity following resection of aneurysms of the aortic arch can be greatly reduced using this simplified technique.

Enlargement of the narrow aortic root and oblique insertion of a St Jude prosthesis.

The aortic root was enlarged and a St Jude Medical valve obliquely inserted in 11 patients. Prosthetic valve sizes at least one, but often two, sizes larger than that which would normally be accommodated by the dimensions of the host aortic annulus were used. Closure of the valve was not affected by obliquity. This simple technique is recommended whenever the host aortic annulus does not allow the insertion of a substitute valve of an appropriate size.

Mitral valve annuloplasty: results in an underdeveloped population.

The long term results of mitral valve annuloplasty for pure mitral regurgitation are assessed. Thirty-three of the 100 patients (mean age 16 years) had acute rheumatic carditis at the time of operation. Three basic techniques were used: measured asymmetrical annuloplasty (Reed) in 37 patients, posterior plication annuloplasty in 56, Carpentier ring in seven. There were six early deaths, two of which were valve related. The mean follow up period for surviving patients was 4.8 (SD 1.2) years (range 3-9 years). Fourteen patients died late, all but one from valve related causes. Twenty-five had reoperation and all these had mitral valve replacement. The actuarial survival with initial repair was 56% at five years and 42% at eight years. Thirteen patients were lost to follow up. Of the remaining 42 patients, only 27 have an excellent or good clinical result. Eleven patients have only a satisfactory result, with a significant degree of residual or recurrent mitral regurgitation or disability or both. Possible causes of valve related failure were investigated; no significant difference was found between age groups or between acute and chronic cases. Poorer results, of statistical significance, were observed in patients in functional class IV and in those whose operation was performed on an emergency basis. No difference could be found between the results of the three techniques of mitral valve annuloplasty used. This retrospective study indicates that conservative procedures which are limited to annular repair produce inferior results. Valvuloplasty has many theoretical advantages which need practical confirmation.

Mitral valvuloplasty: a learning curve.

Failure to appreciate the importance of each of the functional components of the mitral valve has resulted in general disenchantment with the results of mitral valve annuloplasty. Consequently, the technique of mitral valvuloplasty evolved. From January 1981 through March 1982, 100 patients underwent mitral valvuloplasty. The following basic techniques were used: commissurotomy (55 patients), chordal resection (95 patients), shortening (52 patients) and fenestration (16 patients), resection of leaflet tissue (16 patients), and Carpentier-Edwards ring insertion (69 patients). Two consecutive groups of patients were compared. In group 1 (the first 30 patients) there were four early and two late deaths; in group 2 (the remaining 70 patients) there were no early and two late deaths. Three patients in each group required a subsequent valve replacement (p less than .01). Hence, the linear valve failure rate was 12.2% and 4.8% a patient year for groups 1 and 2, respectively. Seventy-one percent of the survivors in group 1 and 84% of those in group 2 had no or only mild residual mitral valve dysfunction. Poor initial results should not discourage surgeons because mitral valvuloplasty has a striking learning curve.