Christos Alexiou

Outcome after repair of tetralogy of Fallot in the first year of life

BACKGROUND The purpose of this study was to evaluate the early and late outcome after repair of tetralogy of Fallot in the first year of life. METHODS Between 1974 and 2000, 89 consecutive infants with a mean age of 6.3 +/- 2.6 months (range, 15 days to 12 months) underwent repair of tetralogy of Fallot (ventricular septal defect and pulmonary stenosis) by one surgeon (J.L.M.). Three infants had previous palliative operations. Sixty-seven procedures were urgent or emergency. A transannular patch was inserted in 69 patients (77.5%). Follow-up was complete, averaging 13.4 +/- 5.6 years (range, 0 to 25.4 years). RESULTS There was one operative death (1.1%). Mean right ventricular to left ventricular pressure ratio postoperatively was 0.4 +/- 1.1 (in 79 patients, < 0.5). Fourteen patients underwent reoperations or reinterventions. There were no reoperations for residual or recurrent ventricular septal defect. Kaplan-Meier freedom from reoperation or reintervention for any cause at 20 years was 85% +/- 4.4%, for relief of right ventricular outflow tract obstruction it was 94% +/- 3.1%, and for pulmonary valve replacement this was 95.4% +/- 2.6%. Use of a transannular patch did not significantly affect the need for reoperation or reintervention. There was one late death (leukemia). Kaplan-Meier 20-year survival was 97.8% +/- 1.9%. On latest echocardiography, 42 patients had moderate pulmonary regurgitation, 4 had a right ventricular outflow tract gradient more than 40 mm Hg, and 86 had good biventricular function. Twelve-lead electrocardiography was performed in all and 24-hour electrocardiography in 61 patients. One patient (1.1%) exhibited late recurrent ventricular tachycardia requiring implantation of a defibrillator. The remaining 86 patients are in New York Heart Association class I with none of them receiving antiarrhythmic medications. CONCLUSIONS These data strongly support the concept of early repair of tetralogy of Fallot. It is associated with an acceptable operative risk and a low incidence of significant arrhythmias, and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and long-term follow-up for their early recognition is essential.

Aortic valve replacement in children: are mechanical prostheses a good option?

OBJECTIVE The choice of the most appropriate substitute in children with irreparable aortic valve lesions remains controversial. The aim of this study was to assess early and late outcomes following aortic valve replacement (AVR) with mechanical prostheses in children. PATIENTS Fifty-six patients (42 male, 14 female, mean age 11.2, range 1-16 years) undergoing AVR with mechanical prostheses between October 1972 and January 1999 were evaluated. Thirty-six patients (64.2%) underwent previous cardiac surgery. Disease aetiology was congenital in 47 patients (congenital aortic stenosis in 33, and other congenital abnormalities in 14) (83.9%), infective in four (7. 1%), rheumatic in two (3.4%), and three (5.3%) had connective tissue disorders. Haemodynamic indication for AVR was aortic regurgitation (AR) in 24 (42.8%), aortic stenosis (AS) in 22 (39.2%) and mixed disease in ten (17.8%). Twenty-eight patients (50.0%) were in New York Heart Association (NYHA) class III-IV before surgery. Concomitant procedures were performed in 31 patients (55.3%), including aortic root enlargement in 28 (50%). The mean size of implanted valves was 22.4 mm (range 17-27 mm). All patients received long-term anticoagulation treatment with sodium warfarin, aiming to maintain an international normalized ratio (INR) between 2.5-3.0. The mean follow-up was 7.3 years (range 0-26, total 405 patient-years). RESULTS Operative mortality was 5.3% (three patients). Three patients developed complete heart block requiring pacing, two of them permanently. Late events included valve thrombosis (one), transient stroke (one), paravalvular leak of a mitral prosthesis (one), aneurysm of sinus of Valsalva (one) and pannus ingrowth (one). There was no major haemorrhagic event. Five patients required re-operation (8.9%), but none due to outgrowth of the valve. Regarding actuarial freedom from thrombo-embolism, any valve-related event and re-operation at 20 years was 93, 86.6 and 86. 4%. There were three late deaths. Actuarial survival, including operative mortality, at 10 and 20 years was 91 and 84.9%. The actuarial survival for the group of the patients with congenital AS (n=33) at 10 and 20 years was 93.5%, whereas for the children with other congenital heart problems (n=14) this was 85.7 and 64.3% (P=0. 09). At the latest clinical evaluation, 44 children were in NYHA class I and six were in class II. The mean gradient across the aortic prosthetic valve on echocardiography was 17.9 mmHg (range 0-47 mmHg). CONCLUSIONS Mechanical AVR, with enlargement of the aortic root if necessary, remains an excellent treatment option in children. It is associated with acceptable operative mortality, low incidence of late events and re-operation, and provides good long-term survival. It clearly represents a good alternative to available biological substitutes, including the pulmonary autograft (Ross procedure).

Mitral valve replacement with mechanical prostheses in children: improved operative risk and survival.

OBJECTIVE The purpose of this study was to assess the early and late outcome following mitral valve replacement (MVR) with mechanical prostheses in children. PATIENTS AND METHODS Between 1981 and 2000, 44 consecutive children (mean age 6.8+/-4.7 years, 2 months--16 years) underwent mechanical MVR in Southampton. Twenty-three children were less than 5-years-old and nine were infants. Disease aetiology was congenital in 37, rheumatic in four, infective in two and Marfans syndrome in one. Mitral regurgitation was present in 36 and mitral stenosis in eight. Concomitant procedures were performed in 13, including aortic valve replacement (AVR) in seven. Follow-up was complete (mean 6.4+/-4.8 years, 1 month--18.1 years). RESULTS The overall operative mortality was 14% (six patients). Before and after 1990 operative mortality was 31 vs 3.6% (P=0.02). From 1990, operative mortality for infants was zero out of six, for children less than 5-years-old was one out of 16 (one death after emergency AVR and MVR) and for older children it was 0/12. Seven children experienced valve or anticoagulation treatment-related events and eight had a mitral valve re-operation. Ten-year freedom from thromboembolism, prosthetic valve infection, bleeding, paravalvular leak and a mitral valve re-operation was 92.8+/-5.2, 97.3+/-2.7, 97.7+/-2.3, 97.2+/-2.7 and 75+/-9.7%, respectively. Overall 10-year survival was 78+/-7% (four late deaths); for children under vs over 5 years it was 61+/-11 vs 95.2+/-4.6% (P=0.02), for atrio-ventricular septal defect (AVSD) vs other pathology 55+/-15 vs 89+/-6.1% (P=0.05) and for those operated before 1990 vs after 1990 it was 63+/-8.1 vs 86+/-8.2% (P=0.04). CONCLUSIONS Mechanical MVR, in the current era, carries a low operative risk across the spectrum of paediatric age. Late survival is better for older children and those having no-AVSD pathology but it has improved substantially during the 1990s irrespective of age and disease aetiology.

Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach

OBJECTIVE The optimal time and approach of repair of tetralogy of Fallot (TOF) remain controversial. The purpose of this study was to evaluate the outcome following repair of TOF in infants with particular regard to the surgical approach used. PATIENTS One hundred and sixty infants (mean age 195+/-89 days, range 11-364 days) undergoing repair of a simple TOF were studied. Between 1974 and 2000, a transventricular approach (RV) was used in 91 and between 1988 and 2000, a transatrial (RA) approach in 69 infants. Ten of these infants (6.2%) had a previous palliative shunt (four in the RV versus six in the RA group). A transannular patch (TAP) was inserted in 96 (60%) infants (76 versus 20). Follow-up was complete (mean 14.5+/-5.2 versus 6+/-1 years). RESULTS There were three operative deaths (1.9%), (two in RV versus one in RA group). A re-operation for right ventricular outflow tract obstruction (RVOTO) was performed in 19 patients (3 versus 16). Ten-year freedom from re-operation for RVOTO (+/-standard error of the mean) was 88+/-4% (98+/-2 versus 72+/-6%, P<0.0001). Within the RA group, 5-year freedom from re-operation for RVOTO for those who had a TAP was 79+/-9% and it was 75+/-4% for those having a simple repair. Six patients in the RV group required pulmonary valve replacement (PVR). Ten-year freedom from PVR was 98+/-1% (97+/-2 versus 100%, P=0.3). There were two late deaths, one in each group. Ten-year survival was 97+/-1%. One patient in the RV group developed late recurrent ventricular tachycardia requiring the implantation of a defibrillator. At most recent echocardiography, all but the patient who had the defibrillator had good right and left ventricular function. CONCLUSIONS Transventricular and transatrial repair of TOF in infancy, are associated with an acceptable operative risk, low incidence of late arrhythmia, good bi-ventricular function and excellent survival. In our experience, however, transatrial repair has a disturbing incidence of early and mid-term residual or recurrent RVOTO, even when a TAP has been used.

Open Commissurotomy for Critical Isolated Aortic Stenosis in Neonates

BACKGROUND The optimal management of critical aortic stenosis in early infancy remains controversial. The aim of this study was to assess the early and late outcomes following open surgical valvotomy for critical aortic stenosis in neonates and to provide a framework of data against which current results of other treatment approaches can be evaluated. METHODS Eighteen consecutive neonates (mean age 9.2 days, range 1 to 26 days) undergoing an open valvotomy for critical isolated aortic stenosis (the standard treatment for this condition in our unit) between 1984 and 2000 were studied. The mean aortic valve gradient was 79.4 mm Hg. Twelve neonates received prostaglandins and 10 received inotropic agents preoperatively. Follow-up was complete (mean 8.1 years, range 1 month to 15 years). RESULTS There was no operative mortality. At discharge, the mean aortic valve gradient was 37.2 mm Hg, with 6 patients having mild and 2 having moderate aortic regurgitation. Six patients required a reoperation; 3 of these had an aortic valve replacement at 9 to 11 years of age. Kaplan-Meier 5- and 10-year freedoms from any aortic reoperation or reintervention were 85 and 55%, respectively; 5- and 10-year freedoms from aortic valve replacement were 100 and 79%, respectively. A 14-year-old boy died from endocarditis 4 years following an aortic valve replacement in another unit. Kaplan-Meier 10-year survival was 100%. All survivors are in New York Heart Association I class and are leading normal lives. Their mean aortic valve gradient is 34.5 mm Hg, and none has significant aortic regurgitation. CONCLUSIONS Open valvotomy for critical aortic stenosis in neonates carries a low operative risk and provides lengthy freedom from recurrent stenosis or regurgitation. Reoperations are inevitable, but aortic valve replacement can be delayed until the implantation of an adult-sized prosthesis is possible. Late survival is excellent. We consider open surgical valvotomy to be the treatment of choice for critical neonatal aortic stenosis.

Aortic root replacement in patients with Marfan’s syndrome: the Southampton experience

BACKGROUND The purpose of this study was to evaluate the early and late clinical outcome after aortic root replacement (ARR) in patients with Marfans syndrome. METHODS A total of 65 consecutive patients with Marfans syndrome (mean age 41.7 +/- 10.7 years, range 15 to 76 years) undergoing ARR between 1972 and 1998 in Southampton were studied. Of the patients, 45 had a chronic aneurysm of the ascending aorta and 20 had a type A dissection (16 acute and 4 chronic). The operations were elective in 38 and nonelective in 27 cases (emergency in 22 and urgent in 5). Mean size of the ascending aorta was 6.3 +/- 1.4 cm (3.8 to 12 cm). A Bentall procedure was performed in 62 and a homograft root replacement in 3 patients. Mean follow-up was 8 +/- 4.1 years (0 to 22.9 years). RESULTS Operative mortality was 6.1% (4 deaths) (for the elective vs nonelective procedures it was 2.6% vs 11%, p = 0.2). The 10-year freedom from thromboembolism, hemorrhage, and endocarditis was 88%, 89.8%, and 98.4% (0.9%, 0.9%, and 0.2% per patient-year) and from late aortic events it was 86.3% (1.3% per patient-year). Aortic root replacement for dissection was an independent predictor of occurrence of late aortic events (p = 0.01). Five patients had a reoperation with one early death. The 10-year freedom from reoperation was 89.2% (1.1% per patient year) (for elective and nonelective procedures, 90.8% vs 84.6%, p = 0.6). The 10-year survival, including operative mortality, was 72.7% (for elective and nonelective procedures, 78% vs 66.5%, p = 0.6). Late aortic events was an independent adverse predictor of survival (p = 0.02). CONCLUSIONS In patients with Marfans syndrome, elective ARR, usually for chronic aneurysm, is associated with a low mortality, low rate of aortic complications, and good late survival. Nonelective ARR, mostly for dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended. Long-term clinical and radiologic follow-up to prevent or to treat late aortic events is highly desirable.

Is there still a place for open surgical valvotomy in the management of aortic stenosis in children? The view from Southampton.

OBJECTIVE The most appropriate management of aortic stenosis (AS) in children remains controversial. The purpose of this study was to determine the outcome following open valvotomy for AS in children. METHODS Ninety-seven consecutive, unselected, children (mean age 3.2 +/- 3.6 years, 1 day--15 years) underwent an open valvotomy for critical (n=36) or severe (n=61) AS between 1979 and 2000 in Southampton. Twenty-six were neonates (1--31 days), 27 were infants (1--12 months) and 44 were older children (1--15 years). Mean follow-up was 10 +/- 5.4 years, 1 month--21.9 years. RESULTS Two neonates died early giving an overall operative mortality of 2.1% (7.7% for the neonates and 0% for infants and older children). The mean aortic gradient was reduced from 76 to 24.5 mmHg (P < 0.0001). Residual or recurrent AS occurred in 17 patients and severe aortic regurgitation in eight patients. Kaplan--Meier 10-year freedom from residual or recurrent AS was 83.1 +/- 4.7% and from severe aortic regurgitation was 95.3 +/- 2.7%. Twenty-five patients required an aortic re-operation or re-intervention, 18 of whom had an aortic valve replacement (AVR) (mean valve size 21.8 +/- 0.9 mm, range 21--25 mm). Ten-year freedom from any aortic re-operation or re-intervention was 78.4 +/- 5.2% and from AVR was 85.1 +/- 4.6%. There were ten late deaths. Overall 10-year survival, including hospital mortality, was 90.2 +/- 3.1% (69.7 +/- 9.7% for the neonates, 92 +/- 5.4% for the infants and 100% for older children, (P < 0.0001). Ten-year survival for children with isolated AS (n = 69) was 100% and for those with associated cardiovascular problems (n = 28) was 67.3 +/- 8.9% (P < 0.0001). All survivors are in New York Heart Association functional class I. CONCLUSIONS Open valvotomy remains the gold standard in the management of AS in neonates, infants and older children. It is associated with low operative mortality and provides lengthy freedom from recurrent AS and regurgitation. Re-operations are common but if AVR is required, implantation of an adult-sized prosthesis is usually possible. There is a late death-hazard for those with severe associated lesions, but the survival prospects for the patients with isolated AS are excellent.

Surgery for infective valve endocarditis in children

OBJECTIVE Surgery for endocarditis in children is relatively uncommon. Our aim is to assess operative mortality, recurrent infection, re-operation and long-term survival rates following surgery for infective valve endocarditis in children. PATIENTS Sixteen consecutive children (ten female, six male, mean age 11.8 years, range 25 days-16 years) undergoing surgery between 1972 and 1999 in Southampton were studied. The aortic valve was affected in five, mitral in four, aortic and mitral in one, tricuspid in five and a pulmonary homograft in one patient. Prosthetic valve endocarditis was present in three. Twelve surgical interventions were emergency and four urgent. Indications for operation included cardiac failure in five, severe valvular dysfunction in nine, vegetations in nine, persistent sepsis in four and embolization in four patients. The offending micro-organism was identified in 13. Valve replacement was performed in 11 and excision of vegetations in two and excision of vegetations and repair in three. Follow-up was complete (mean 11.2 years, range 2 months to 26.3 years, total 179.5 patient years). RESULTS There was one operative death (6.2%) in a 25-day-old neonate who presented in a moribund condition. Endocarditis recurred in one patient (6.25%). Freedom from recurrent infection at 10 and 20 years was 100.0 and 87.5%. Seven surgical re-interventions were required in four (25.0%) patients with no operative mortality. Freedom from re-operation at 1, 5, 10 and 20 years, was 84.6, 76.1, 76.1 and 60.9%, respectively. Two patients died 15 and 23 years after their first operation. The cause of the late deaths was non-cardiac in the first and unknown in the other. Actuarial survival, including operative mortality, at 1, 15 and 20 years was 93.7, 93.7 and 78.1%. CONCLUSIONS Surgery in children with infective valve endocarditis can be performed with low operative mortality. Although some patients may require re-operation, freedom from recurrent infection and long-term survival are satisfactory.

Mitral valve repair for active culture positive infective endocarditis

Objective: To describe the clinical and echocardiographic outcome after mitral valve (MV) repair for active culture positive infective MV endocarditis. Patients and methods: Between 1996 and 2004, 36 patients (mean (SD) age 53 (18) years) with positive blood culture up to three weeks before surgery (or positive culture of material removed at operation) and intraoperative evidence of endocarditis underwent MV repair. Staphylococci and streptococci were the most common pathogens. All patients had moderate or severe mitral regurgitation (MR). Mean New York Heart Association (NYHA) class was 2.3 (1.0). Follow up was complete (mean 38 (19) months). Results: Operative mortality was 2.8% (one patient). At follow up, endocarditis has not recurred. One patient developed severe recurrent MR and underwent valve replacement and one patient had moderate MR. There were two late deaths, both non-cardiac. Kaplan-Meier five year freedom from recurrent moderate to severe MR, freedom from repeat operation, and survival were 94 (4)%, 97 (3)%, and 93 (5)%, respectively. At the most recent review the mean NYHA class was 1.17 (0.3) (p < 0.0001). At the latest echocardiographic evaluation, left atrial diameters, left ventricular end diastolic diameter, and MV diameter were significantly reduced (p < 0.05) compared with preoperative values. Conclusions: MV repair for active culture positive endocarditis is associated with low operative mortality and provides satisfactory freedom from recurrent infection, freedom from repeat operation, and survival. Hence, every effort should be made to repair infected MVs and valves should be replaced only when repair is not possible.

How I do it: transapical cannulation for acute type-A aortic dissection

Aortic dissection is the most frequently diagnosed lethal disease of the aorta. Half of all patients with acute type-A aortic dissection die within 48 hours of presentation. There is still debate as to the optimal site of arterial cannulation for establishing cardiopulmonary bypass in patients with type-A aortic dissection.Femoral artery cannulation with retrograde perfusion is the most common method but because of the risk of malperfusion of vital organs and atheroembolism related to it different sites such as the axillary artery, the innominate artery and the aortic arch are used. Cannulation of these sites is not without risks of atheroembolism, neurovascular complications and can be time consuming. Another yet to be popularised option is the transapical aortic cannulation (TAC) described in this article. TAC consists of the insertion of the arterial cannula through the apex of the left ventricle and the aortic valve to lie in the sinus of Valsalva. Trans-oesophageal guidance is necessary to ensure correct placement of the cannula.TAC is an excellent method of establishing cardiopulmonary bypass as it is quick, provides a more physiological method of delivering antegrade arterial flow and is the only method to assure perfusion of the true lumen.