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Artificial Organs

33(11):915–921, Wiley Periodicals, Inc.


© 2009, Copyright the Authors
Journal compilation © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

Mechanical Aortic Valve Replacement in Children


and Adolescents After Previous Repair of Congenital
Heart Disease

*Aron-Frederik Popov, *Kasim Oguz Coskun, *Theodor Tirilomis, *Jan Dieter Schmitto,
†José Hinz, ‡Thomas Kriebel, *Friedrich Albert Schoendube, and *Wolfgang Ruschewski
*Department of Thoracic Cardiovascular Surgery; †Department of Anaesthesiology, Emergency and Intensive
Care Medicine; and ‡Department of Pediatric Cardiology and Intensive Care Medicine, University of Göttingen,
Göttingen, Germany

Abstract: Due to improved outcome after surgery for was 23 mm (range 17–29 mm). There were neither early
congenital heart defects, children, adolescents, and grown- deaths nor late mortality until December 2008. Reopera-
ups with congenital heart defects become an increasing tions were necessary in five (33%) and included implan-
population. In order to evaluate operative risk and early tation of a permanent pacemaker due to complete
outcome after mechanical aortic valve replacement atrioventricular block in two (15%), mitral valve replace-
(AVR) in this population, we reviewed patients who ment with a mechanical prosthesis due to moderate to
underwent previous repair of congenital heart defects. severe mitral regurgitation in one (7%), aortocoronary
Between July 2002 and November 2008, 15 (10 male and bypass grafting due to stenosis of a coronary artery in one
5 female) consecutive patients (mean age 14.5 ⫾ 10.5 (7%), and in one (7%), a redo subaortic stenosis resection
years) underwent mechanical AVR. Hemodynamic indica- was performed because of a secondary subaortic stenosis.
tions for AVR were aortic stenosis in four (27%), aortic At the latest clinical evaluation, all patients were in good
insufficiency in eight (53%), and mixed disease in three clinical condition without a pathological increased
(20%) after previous repair of congenital heart defects. gradient across the aortic valve prosthesis or paravalvular
All patients had undergone one or more previous cardio- leakage in echocardiography. Mechanical AVR has excel-
vascular operations due to any congenital heart disease. lent results in patients after previous repair of congenital
Concomitant cardiac procedures were performed in all of heart defects in childhood, even in combination with
them. In addition to AVR, in two patients, a mitral valve complex concomitant procedures. Previous operations
exchange was performed. One patient received a right do not significantly affect postoperative outcome.
ventricle-pulmonary artery conduit replacement as con- Key Words: Reoperation—Mechanical aortic valve
comitant procedure. The mean size of implanted valves replacement—Congenital heart disease.

Despite steady improvements in the results of increasing challenge for pediatric cardiologists and
cardiac surgery, there has been a trend in operating cardiac surgeons, as improved diagnostic and thera-
on higher-risk patients, which leads to increased mor- peutic tools allow the majority of newborns with con-
bidity and mortality. Children and adolescents, after genital heart disease to survive to adulthood.
previous repair of congenital heart disease, are an The grown-ups with congenital heart defects
(GUCH) are a very inhomogeneous group of
patients with a broad spectrum of different diagnoses
doi:10.1111/j.1525-1594.2009.00886.x
with variable morphology and pathophysiology.
Received May 2009; revised June 2009. Many GUCH, however, require reoperations for
Address correspondence and reprint requests to Dr. Aron-
Frederik Popov, Department of Thoracic and Cardiovascular definitive corrective repair, residual and or new
Surgery, University of Göttingen, Robert-Koch-Strasse 40, 37099 lesions, additional palliative procedures, and some of
Göttingen, Germany. E-mail: popov@med.uni-goettingen.de them need replacement of valves.
Presented in part at the 5th International Conference Pediatric
Mechanical Circulatory Support Systems & Pediatric Cardiopul- Aortic valve replacement (AVR) represents one of
monary Perfusion held May 27–30, 2009 in Dallas, TX, USA. several treatment options currently available for

915

aor_886 915..921
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916 A.-F. POPOV ET AL.

children, adolescents, and GUCH presenting with assessed by the determination of the shortening frac-
severe aortic valve (AV) lesions not suitable for tion and was within normal ranges in most patients
valve-sparing surgical procedures. However, AVR is (shortening fraction, 37% ⫾ 6.1; range, 25% to 47%).
associated with specific disadvantages. Bioprostheses Measurements of left ventricular end-diastolic
are considered unsuitable for children due to their diameter were available in all patients (median,
early degeneration (1), whereas homografts for AVR 48 ⫾ 8.4 mm; range, 31 to 61 mm).The LV end-systolic
that provide excellent hemodynamics are resistant diameter was found to be normal in all patients
to infection and allow implantation even in small (median, 30 ⫾ 4.5 mm; range, 23 to 40 mm).
patients. However, their early degeneration is com- Genetic malformations affecting the heart, aorta,
parable to that of bioprostheses and thus constitutes and other major vessels were seen in seven patients.
a major drawback (2). Pulmonary autografts, used in One patient (7%) had Shone’s complex, one (7%)
the Ross procedure, do not degenerate and have a had Turner syndrome, one (7%) had Smith–Lemli–
growth potential (3), but this procedure creates a Opitz syndrome, one (7%) Rubinstein–Taybi syn-
new pathology on the right ventricular outflow tract drome, one (7%) had DiGeorge syndrome, one (7%)
(RVOT), and the fate of the pulmonary valve is had Williams–Beuren syndrome, and one (7%) had
still uncertain. The use of a mechanical prosthesis, Noonan syndrome documented by chromosomal
however, is often criticized because of possible com- analysis. Preoperative characteristics of the patients
plications, inconvenience from anticoagulation (4), at the time of AVR are shown in Table 1.
and need for reoperation because of outgrowth.
The purpose of the present study was to review the Previous cardiac surgical procedures
experience with mechanical AVR in patients with All patients had undergone previous cardiac surgi-
previous repair of congenital heart defects retrospec- cal procedures due to congenital heart disease with a
tively and to evaluate early and midterm survival variety of abnormalities. They had undergone a total
rates, morbidity, and complication. of 31 cardiac surgical operations (at our institution or
another) with an average of 2.1 ⫾ 1.3 surgical inter-
PATIENTS AND METHODS ventions per patient before AVR. Details are summa-
rized in Table 2.
Study population
Between July 2002 and November 2008, 15 con- Surgical procedure
secutive patients (median age 14.5 ⫾ 10.5 years, Midline resternotomy was performed and standard
range 4.1 month–40.3 years) underwent AVR with a cardiopulmonary bypass (CPB) with systemic (18–
mechanical prosthesis at our institution. Five were 28°C) hypothermia, established via ascending
female patients and 10 were male. All patients had aortic and single atrial or bicaval cannulation, and
undergone one or more previous cardiovascular for myocardial protection crystalloid cardioplegia
operations due to congenital heart disease. (Brettschneider’s solution) were used in all cases. The
Before AVR, two (13%) had undergone percuta- individual AV annulus was measured at surgery using
neous AV interventions, 15 (100%) had undergone at calibrated Hegar dilators. All mechanical prostheses
least one previous surgical intervention, 10 (67%) were positioned in the subcoronary position using an
had undergone two previous surgical interventions, Ethibond-interrupted suture technique. In all cases, a
and two (13%) had undergone three previous surgi- mechanical St. Jude Medical valve was implanted.
cal interventions to address AV lesions, other cardiac Transesophageal echocardiographic control was
lesions, or both. The mean age at first operation was performed before protamine administration and
2.5 ⫾ 4.8 years (range, 0.1–17), 5.4 ⫾ 3.1 years (range, removal of cannulas to confirm adequate valve func-
0.4–12) at the second operation, and 7 ⫾ 0 years tion and complete air evacuation.
(range, 0.4–7) at the third operation.
All patients in the study had two-dimensional Definitions
echocardiograms with Doppler studies and cardiac Operative mortality includes death occurring
catheterization before AVR. Echocardiography within 30 days after the operation or during the same
evaluation was reported as the mean value ⫾ hospital admission. Deaths taking place more than 30
standard deviation. Hemodynamic indications for days postoperatively following the patient discharge
AVR were aortic stenosis in four (27%), aortic insuf- from hospital are termed late deaths. Freedom
ficiency in eight (53%), and mixed disease in three from cardiovascular events contains thromboembo-
(20%) after previous repair of congenital heart lism, anticoagulation treatment bleeding, any valve-
defects. Left ventricular (LV) systolic function was related event, and any reoperation.

Artif Organs, Vol. 33, No. 11, 2009


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MECHANICAL AORTIC VALVE REPLACEMENT IN CHILDREN, ADOLESCENTS, AND GUCH 917

TABLE 1. Clinical characteristics


No. or Percentage
Patient characteristics mean ⫾ SD (%) or range
No. of patients 15
Age (years) 14.9 ⫾ 10.5 0.3–40
Sex
Male 10 67
Female 5 33
Previous cardiac operation
One previous operation 15 100
Two previous operations 10 67
Three previous operations 2 13
Age at previous operation
1st operation 2.5 ⫾ 4.8 0.1–17
2nd operation 5.4 ⫾ 3.1 0.4–12
3rd operation 7⫾0 0.4–7
Aortic valve lesion
Stenosis 4 27
Insufficiency 8 53
Mixed 3 20
Echocardiography data preoperative
LVEDD (mm) 48 ⫾ 8.4 31–61
LVESD (mm) 30 ⫾ 4.5 23–40
FS (%) 37 ⫾ 6.1 25–47
Genetic cardiovascular anomalies (syndromes) 7 47

LVEDD, left ventricular end-diastolic dimension; LVESD, left ventricular end-systolic


dimension; FS, fractional shortening.

Anticoagulation graphy was performed by a pediatric cardiologist


All patients were given phenprocoumon orally, postoperatively before discharge from our hospital.
aiming to maintain a closely monitored international The patients were then examined by the referring
normalized ratio (INR) within the range of 2.5 to 3.0 cardiologist at regular intervals or in our institution
for AVR and 2.5 to 3.5 for mitral valve replacement by a pediatric cardiologist. The echocardiographic
(MVR). data for the study were taken from the cardiologists’
report.
Follow-up
No patients were lost to follow-up. Follow-up time Statistical analysis
ranged from 2 to 66 months (mean 30 months, cumu- Statistical analysis was performed by using com-
lative 488 patient months). Transthoracic echocardio- mercial statistic software (Statistica 5.1, StatSoft, Inc.,

TABLE 2. Previous cardiac surgical procedures


Number of Percentage
Previous operations patients, mean ⫾ SD (%)
Aortic valve commissurotomy 5 16
Resection of subaortic stenosis 4 13
Repair of supravalvular stenosis 4 13
Repair of coarctation aorta 2 6.5
Repair of aortic valve 1 3
Ventricular septal defect closure 2 6.5
Intraventricular tunnel repair 1 3
Complete atrioventricular septal defect repair 2 6.5
Mitral valve replacement 1 3
Pulmonary valve replacement (Homograft) 1 3
Pulmonary valve replacement 2 6.5
Correction of TGA 2 6.5
Correction of Fallot 2 6.5
Balloon aortic valvuloplasty 2 6.5
Total 31 100
Average per patient 2.1 ⫾ 1.3

TGA, transposition of great arteries.

Artif Organs, Vol. 33, No. 11, 2009


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918 A.-F. POPOV ET AL.

Tulsa, OK, USA). Freedom from time-related events Complications and reoperations
was calculated using the product-limit method of At follow-up, midterm data (30 ⫾ 23 months) could
Kaplan–Meier. be obtained for all patients (100%). Quality of life was
considered normal in 14 (93%), and severely dimin-
RESULTS ished in one (7%). A thromboembolic event was
observed in one patient. This patient suffered from a
Operative results stroke and lives in a care facility for children.
All patients survived the operation. The mean size Reoperation was necessary in five (33%) and
of implanted valves was 23 mm (range, 17–29 mm). In included implantation of a permanent pacemaker due
all cases, 27 concomitant procedures were performed to complete atrioventricular block in two (15%),
with an average of 2 ⫾ 1.3 (range, 1–3) per patient. MVR with a mechanical prosthesis due to moderate
These were implantation of a composite mechanical to severe mitral regurgitation in one (7%), aortocoro-
prosthesis in one, resection of subaortic stenosis nary bypass grafting due to stenosis of a coronary
(SAS resection) in five, an ascending aortic enlarge- artery in one (7%), and in one (7%), a redo subaortic
ment in four, an aortic replacement in one, closure of stenosis resection was performed because of a second-
a ventricular septal defect in two, closure of an atrial ary subaortic stenosis. No complications occurred
septal defect in one, a conduit replacement in two, because of multiple redo sternotomies due to previous
RVOT procedures in one, mitral valve procedures cardiac surgical procedures. No patients had mechani-
(including MVR) in two, pulmonary valve proce- cal valve endocarditis. There were no hemorrhagic
dures (including pulmonary valve replacement) in events such as gastrointestinal or cerebral bleeding
two, a tricuspid valve procedure in one, coronary pro- observed during follow-up. Kaplan–Meier freedoms
cedures in two, and miscellaneous procedures in from any cardiovascular events (including cardiologi-
three. The median CPB time at surgery was 259 min cal or surgical reintervention) at 60 months were 52%
(range, 100–411 min), and the median aortic cross (Fig. 1).
clamp time was 142 min (range, 60–227 min). The
mean body core temperature was 23 ⫾ 4°C (range, Echocardiography
18–28°C). The median intensive care unit stay was Postoperative echocardiography at follow-up was
15 ⫾ 34 days (range, 1–133 days) and the median hos- performed in all patients. Echocardiographic data are
pital stay was 30 ⫾ 35 days (range, 11–150 days). All summarized in Table 4. Fractional shortening of the
details are summarized in Table 3. left ventricle was normal in all patients (35% ⫾ 10.6;

TABLE 3. Operations data and surgical procedures at the time of mechanical


aortic valve replacement
Range or % of
n = 15 Mean ⫾ SD procedures
Cardiopulmonary bypass time (min) 245 ⫾ 29 100–411
Aortic cross clamp time (min) 142 ⫾ 24 60–227
Body core temperature (°C) 23 ⫾ 4 18–28
Prosthesis size (mm) 23 17–29
Concomitant procedures
Composite mechanical prosthesis 1 3.7
SAS resection 5 18.5
Ascending aortic enlargement 4 14.8
Aortic replacement 1 3.7
Closure of residual VSD 2 7.4
Closure of ASD 1 3.7
Conduit replacement 2 7.4
RVOT procedures 1 3.7
Mitral valve procedures 2 7.4
Pulmonary valve procedures 2 7.4
Tricuspid valve procedures 1 3.7
Coronary procedures 2 7.4
Miscellaneous 3 11.1
All operations (concomitant procedures) 27 100
Average per patient 2 ⫾ 1.3 1–3
ICU stay (days) 15 ⫾ 34 1–133
Hospital stay (days) 30 ⫾ 35 11–150

SAS, subaortic stenosis; VSD, ventricular septal defect; ASD, atrial septal defect; RVOT,
right ventricular outflow tract; ICU, intensive care unit.

Artif Organs, Vol. 33, No. 11, 2009


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MECHANICAL AORTIC VALVE REPLACEMENT IN CHILDREN, ADOLESCENTS, AND GUCH 919

DISCUSSION
(% ) Freedom from cardiovascular events

100
As a result of increasing survival rates for children
80 with congenital heart disease over the last decades, a
new patient population of adults with congenital
60
heart disease is emerging today.
Patients with both native unoperated and operated
40
malformations contribute to the GUCH population.
20
Survivors without surgical treatment mainly have
simple malformations, but a few have complex dis-
0 eases, and some have survived with secondary cardiac
0 10 20 30 40 50 60 lesions. Among operated malformations, there are
Follow up (months) patients with a “complete” repair (anatomical and
Number at risk physiological), others with a definitive palliation
15 12 8 6 6 4 1
(physiological repair), and some with a simple
FIG 1. Kaplan–Meier freedoms from any cardiovascular events palliation. The clinical spectrum is obviously diversi-
(including cardiological or surgical reintervention) at 60 months fied, depending on the underlying anomaly, surgical
were 52%.
outcome, presence of residua, sequelae and/or com-
plications, length of follow-up, and comorbidities.
range, 24% to 45%). Measurements of left ventricu- However, reports of experiences with reoperations
lar end-diastolic diameter were in normal ranges for congenital heart disease are rare.
(51 ⫾ 5.1 mm, median; range, 42–61 mm). The LV In some cases, children, adolescents, and GUCH
end-systolic diameter was found to be normal in all require reoperations for definitive corrective repair,
patients (median, 34 ⫾ 5.4 mm; range, 27–42 mm). residual and/or new lesions, and additional palliative
Four patients had mild AV regurgitation and two procedures. Others need replacement of valves if
patients showed a mild mitral regurgitation without reconstruction is impossible or fails. The selection of
hemodynamic significance. Transvalvular gradients the most appropriate substitute in those with irrepa-
were within a clinically acceptable range and no para- rable AV lesions remains controversial.
valvular leakage in echocardiography was found. Potential complications after implantation of
mechanical valve prosthesis are thrombosis, hemor-
Midterm survival and functional status rhage, cerebral embolism, endocarditis, ventricular
There was no late death.Two patients showed a mild arrhythmias, and ventricular failure. Repeated valve
mitral regurgitation without hemodynamic signifi- replacement will be needed in some cases during
cance. At the latest clinical examination, all patients childhood because of somatic growth (5). However,
were in New York Heart Association (NYHA) func- although the Ross procedure has growth potential
tional class I leading normal or near-normal lives. and does not require long-term anticoagulation,

TABLE 4. Latest follow-up


Number of patients
(%) or mean ⫾ SD Range
Survival 15 (100)
Reoperation 5 (33)
Mitral valve replacement 1 (7)
Coronary artery bypass grafting 1 (7)
Redo SAS resection 1 (7)
Pacemaker implantation 2 (15)
Thomboembolic events 1 (7)
Hemorrhagic events 0
Echocardiography data postoperative
LVEDD (mm) 51 ⫾ 5.1 42–61
LVESD (mm) 34 ⫾ 5.4 27–42
FS (%) 35 ⫾ 10.6 24–45

SAS, subaortic stenosis; LVEDD, left ventricular end-diastolic dimension; LVESD, left ven-
tricular end-systolic dimension; FS, fractional shortening.

Artif Organs, Vol. 33, No. 11, 2009


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920 A.-F. POPOV ET AL.

the procedure is technically demanding, and late one case, we found subaortic stenosis in a patient
autograft failure, aortic root dilatation, and homo- who required a second operation after mechanical
graft reoperations are common (6–8). AVR. It has been reported after surgical repair of
Moreover, AV repair is an attractive alternative to several congenital heart defects that a recurrent or
valve replacement due to durability of the native AV, residual subaortic stenosis can occur. That is a poten-
good hemodynamics, and avoiding anticoagulation tial critical lesion requiring close follow-up and, even-
therapy (9). However, more studies comparing repair tually, reintervention (18). We surgically address
and replacement of the AV are needed in order to this lesion when the systolic peak gradient reaches
elucidate which groups of patients are suitable for 50 mm Hg, or with any evidence of AV insufficiency
each type of procedure. detected by echocardiography to avoid progressive
Our study reports a single institution’s experience left ventricular hypertrophy and dilatation (19). Our
with AVR using mechanical valves in 15 children who incidence of postoperative complete heart block
underwent previous repair of congenital heart requiring a permanent pacemaker implantation
defects. The absence of early or late mortality in this (3.7%) is similar to those found in the literature
study is most gratifying and reflects improvements (12,13). Moreover, one MVR with a mechanical pro-
made in cardiac surgical technique, preoperative sthesis due to moderate to severe mitral and one
clinical diagnostics, and postoperative intensive care. aortocoronary bypass grafting due to stenosis of a
It compares well with the lowest reported mortality coronary artery were performed after mechanical
rates for mechanical valve replacement in pediatric AVR. However, this miscellaneous group of children,
patients of 0 to 10% for AVR (10–12), although our adolescents, and GUCH is so heterogenous that it is
series contains even GUCH. However, it is difficult to difficult to draw conclusions from the data. There are
compare our results with the rare comparative data so many complex patients in our series that it is not
available from the literature because most published surprising that the morbidity was 33% at latest
series present mixed populations with first and re- follow-up. Otherwise, other potential complications
operations or with different cardiac anomalies. For after mechanical AVR such as bleeding complica-
example, Berdat et al. (13) had 7.6% early mortality tions, endocarditis, or rereplacement of the aortic
in their study of reoperations in GUCH; Monroe prosthesis due to somatic growth were not yet
et al. (14) had an overall operative mortality of 6.9% observed in our series.
in only children over a 25-year period; Klcovansky Anticoagulant therapy is an important issue. Most
et al. (15) had an overall mortality of 1.3% in GUCH of the reports arguing against the use of mechanical
over an 8-year period; and Luciani et al. (16) had an valves mention either no anticoagulant therapy or
excellent survival rate of 99% in adults with congeni- inadequate prophylaxis such as aspirin or aspirin and
tal heart disease over a 5-year period. dipyramidol. Only those studies that employ couma-
Recently, a large European multicenter study (17) din anticoagulant therapy, maintaining an INR of
showed a hospital mortality of 4.1 % for GUCH in a more than 2.0, report absence of thromboembolic
subgroup analysis of 2012 adult patients. incidents. After AVR with a mechanical valve,
Actuarial freedom from any cardiovascular events adequate antithrombotic therapy is mandatory. We
(including cardiological or surgical reintervention) at advocate maintaining an INR of 2.5–3.0 for AVR and
60 months was 52%. However, we did not classify the 2.5–3.5 for MVR. The risk of thromboembolic and
cardiovascular events in different groups because our bleeding complications in patients on oral anticoagu-
sample size was too small. Therefore, “any cardiovas- lant therapy for mechanical heart valves has been
cular event” comprises thromboembolism, anticoagu- thoroughly analyzed by Cannegieter et al. (20). The
lation treatment bleeding, any valve-related event, linear risk of cerebral emboli was 0.68 per 100 patient
and any reoperation. Luciani et al. (16) demonstrated years, while the risk of peripheral emboli was much
that AV surgery in adults with congenital heart lower at 0.03 per 100 patient years. The low incidence
disease can be performed safely with low mortality of the latter is most likely due to lack of detection. In
and with 98% freedom from reoperation at 5 years. our series, only one patient had a thromboembolic
In our series, reoperation (including implantation of event. This patient suffered from a stroke presumably
a permanent pacemaker due to complete atrioven- due to inadequate anticoagulant therapy and lives in
tricular block) after AVR was necessary in five (33%) a care facility for children.
and was also acceptable. The echocardiographic findings showed that four
Our data are almost identical to those reported by patients had mild AV regurgitation (physiological
Alexiou and associates (5) and Arnold and cowork- backflow at the prosthesis) and two patients showed
ers (12) concerning the cardiovascular events. In a mild mitral regurgitation without hemodynamic

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MECHANICAL AORTIC VALVE REPLACEMENT IN CHILDREN, ADOLESCENTS, AND GUCH 921

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Artif Organs, Vol. 33, No. 11, 2009

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