We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction

Anita J. Moon-Grady

Anthony Azakie

BW McCrindle

C Salih

DB McElhinney

GK Sharland

JJ Smolich

K Makikallio

P Eghtesady

Phillip Moore

RK Han

SC Brown

SM Emani

T Shinkawa

English

PubMed

Springer - Publisher Connector

CASE REPORT
Ross-Konno and Endocardial Fibroelastosis Resection
After Hybrid Stage I Palliation in Infancy: Successful Staged
Left-Ventricular Rehabilitation and Conversion to Biventricular
Circulation After Fetal Diagnosis of Aortic Stenosis
Anita J. Moon-Grady • Phillip Moore •
Anthony Azakie
Received: 24 August 2010 / Accepted: 19 September 2010 / Published online: 24 November 2010
 The Author(s) 2010. This article is published with open access at Springerlink.com
Abstract We report a patient who presented during fetal
life with severe aortic stenosis, left-ventricular dysfunction,
and endocardial fibroelastosis (evolving hypoplastic left
heart syndrome). Management involved in utero and
postnatal balloon aortic valvuloplasty for partial relief of
obstruction and early postnatal hybrid stage I palliation
until recovery of left-ventricular systolic function had
occurred. The infant subsequently had successful conver-
sion to a biventricular circulation by combining resection
of endocardial fibroelastosis with single-stage Ross-Konno,
aortic arch reconstruction, hybrid takedown, and pulmon-
ary artery reconstruction.
Keywords Aortic stenosis  Fetal balloon aortic
valvuloplasty  Fetal intervention  Hypoplastic left heart
syndrome  Resection of endocardial fibroelastosis 
Stage 1 hybrid
Introduction
The morphologic and functional spectrum of the left ven-
tricle in severe aortic stenosis ranges from the smaller,
hypertrophied ‘‘borderline’’ left ventricle to the dilated and
thin-walled left ventricle. In addition, several studies have
demonstrated that aortic stenosis with left-ventricular dys-
function presenting in fetal life may lead to hypoplastic left
heart syndrome at birth, with varying degrees of chamber
hypoplasia and endocardial fibroelastosis (EFE) [6, 8, 10].
Neonates with severe aortic stenosis generally require either
surgical valvotomy or transcatheter balloon valvuloplasty
[7], whereas those with hypoplastic left heart are generally
managed with univentricular palliation (Norwood or hybrid
transcatheter-surgical techniques) or cardiac transplantation.
Case Report
A 3.48-kg male infant was delivered by way of planned
repeat Cesarean section at 39 2/7 weeks’ gestation to a
36 year-old gravida 3, para 2 mother. The pregnancy was
complicated by a diagnosis of fetal aortic stenosis made at
22 3/7 weeks’ gestation (Fig. 1). At 23 4/7 weeks’ gesta-
tion, fetal balloon aortic valvuloplasty was performed. After
the procedure, as demonstrated by color Doppler, aortic
arch flow had gone from retrograde to antegrade across the
aortic valve and in the ascending and transverse aortic arch;
there was mild aortic insufficiency. Postnatally the left
ventricle and mitral valve were of normal size; the aortic
valve was mildly hypoplastic at 5 mm with a peak Doppler-
derived gradient of 60 mmHg; and there was significant
left-ventricular hypertrophy (diastolic left ventricular pos-
terior wall dimension, LVPWd 6 mm) with poor systolic
function (LVEF% \ 20%). Doppler flow in the transverse
arch and isthmus was antegrade. Cardiac catheterization
showed increased left atrial pressure of 25 mm Hg. The
aortic valve was dilated by way of a retrograde approach
with a 5-mm Slalom balloon catheter (Cordis Corporation,
Miami, FL, USA) with a decrease in peak-to-peak gradient
from 60 to 45 mm Hg. After valvuloplasty, a 5-mm stent
A. J. Moon-Grady  P. Moore  A. Azakie (&)
Pediatric Heart Center, UCSF Benioff Children’s Hospital,
University of California San Francisco, San Francisco,
CA 94143-0117, USA
e-mail: tony.azakie@ucsfmedctr.org
A. J. Moon-Grady
Fetal Treatment Center, UCSF Benioff Children’s Hospital,
University of California San Francisco, San Francisco,
CA 94143-0570, USA
123
Pediatr Cardiol (2011) 32:211–214
DOI 10.1007/s00246-010-9841-3
was placed in the interatrial septum. The neonate was
maintained on prostaglandin infusion.
On day of life 14, with no change in clinical status or
LVEF%, the child underwent stage I hybrid palliation with
placement of an 18-mm ductal stent and bilateral pul-
monary artery banding. Repeat dilation of the aortic valve
was performed at that time, resulting in a decrease in peak-
to-peak gradient to 11 mm Hg and mild to moderate aortic
insufficiency.
The LVEF% improved gradually to 60% during the sub-
sequent months. Cardiac catheterization at 9 months of age
showed a 38 mm Hg peak-to-peak gradient across the aortic
valve and mild to moderate aortic insufficiency. Left atrial
pressure was 11 mm Hg; left ventricular end diastolic pressure
was 14 mm Hg; and estimated pulmonary vascular resistance
was 4 Woods units. After recovery from a severe intercurrent
pulmonary illness, the child underwent a Ross-Konno pro-
cedure, placement of a 16-mm right ventricle-to-pulmonary
artery homograft, ductus arteriosus and atrial stent removal,
aortic arch reconstruction, pulmonary artery debanding,
resection of extensive EFE (Fig. 2), and closure of an atrial
septal defect. He was successfully extubated on postoperative
day 7 and weaned from inotropic and vasodilatory support.
Follow-up echocardiography showed no evidence of mitral
inflow obstruction or regurgitation, normal left-ventricular
systolic function, and a well-functioning autograft with no
aortic arch obstruction. At catheterization 1 month later, the
cardiac index was 3.3 ml/min/M2 by thermodilution; wedge
pressure mean was 10 mm Hg; and pulmonary vascular
resistance was 6 Woods units (decreasing to 2.6 Woods units
in response to inhaled nitric oxide).
At 1 year of life, left heart dimensions are normal
(mitral valve 13.7 mm [Z score ?0.7], LV end diastolic
diameter 24.9 mm [Z score ?0.3], and LVPWd 6 mm
[Z score ?2.4]), and his systolic function is normal with an
LVEF% of 69% by Simpson’s biplane method (Fig. 3).
Fig. 1 Fetal echocardiogram at
22 4/7 weeks’ gestation. The
left ventricle is dilated with
echo-bright endocardium
suggestive of endocardial
fibroelastosis (a), and there is
left-to-right flow across the
atrial septum (b). Sagittal two-
dimensional (c) and color
Doppler (d) images demonstrate
retrograde perfusion of the
aortic arch in red (arrowheads),
and antegrade flow in the ductal
arch is also apparent (blue).
LA left atrium, LV left ventricle,
RA right atrium, RV right
ventricle
212 Pediatr Cardiol (2011) 32:211–214
123
Discussion
Experimental models involving surgically created aortic
obstruction do not recapitulate the human phenotype [3];
therefore, study of the pathophysiology of this disease has
been difficult. However, natural history studies have shown
that in the presence of certain physiologic findings (spe-
cifically left-to-right atrial shunting and retrograde aortic
arch perfusion in fetal life), progression to hypoplastic left
heart syndrome at term can be reliably predicted [6]. The
rationale for fetal aortic valvuloplasty is to relieve aortic
obstruction and promote antegrade flow through the left
heart and aorta. With restoration of antegrade flow, we
observed continued growth of the left heart structures
through the remainder of gestation; however, ventricular
function did not improve appreciably. The reason for this is
speculative, but it has been proposed that the fetal left
ventricle responds to obstruction with increase in mass
without increase in capillary density, which may result in a
hypertrophied myocardium with increased susceptibility to
ischemia [9, 12]. The extensive EFE in this patient as well
as the observed hypertrophy might support this theory. Fetal
valvuloplasty partially relieved the obstruction, and after
birth we undertook a combined approach that may have
further mitigated the effect of long-standing obstruction and
ischemic insult through relief of obstruction (by way of
additional valvuloplasties) and decrease in preload (left
atrial decompression with atrial stenting). Facilitating
antegrade perfusion of the aorta and coronary circulation
with normally saturated blood, along with improvement in
myocardial perfusion, may have contributed to the slow
improvement in the patient’s systolic and diastolic function.
Previous reports have also documented the possibility of
reversible severe left-ventricular dysfunction and ventricu-
lar growth in the neonate [1, 2, 5]. Emani et al. [4] reported a
series of patients with borderline left heart disease who
underwent ‘‘primary left-ventricular rehabilitation’’ at a
median age of 5.6 months, with aortic and/or mitral valve
surgery and EFE resection, with good short-term results and
no deaths at a median follow-up of 25 months. Our case,
however, represents the first report to our knowledge of a
patient initially palliated with a hybrid transcatheter-surgi-
cal approach after fetal intervention followed by successful
surgical takedown of the hybrid and conversion to biven-
tricular circulation by adding Ross-Konno procedure and
aortic arch reconstruction to the EFE resection.
Fig. 2 Surgical specimens of
the explanted aortic valve (inset,
left) and left-ventricular
endocardium. The aortic valve
was severely dysplastic,
doming, and unicuspid. At the
time of biventricular repair, the
valve was detached from the
aortic wall. The endocardium
was diffusely thickened and
excised from base to apex
through the left-ventricular
outflow tract
Fig. 3 Echocardiogram 2 months after biventricular repair demon-
strates an apex-forming left ventricle and mild residual left-ventric-
ular hypertrophy
Pediatr Cardiol (2011) 32:211–214 213
123
Long-term outcome for patients undergoing extensive
EFE resection as part of left-ventricular rehabilitation are
unknown. Medium-term results from patients undergoing
neonatal Ross-Konno procedures have been mixed, and
particularly poor results in patients requiring concomitant
aortic arch reconstruction have been noted. However, a
recent large single-center experience [11] suggested that
overall survival and functional status may represent
improvement compared with single-ventricle palliation or
neonatal transplantation. Certainly long-term follow-up
data, including evaluation of functional and neurodevel-
opmental status and comorbidities (including pulmonary
vascular disease) will be needed before there is widespread
adoption of this type of approach. However, feasibility of
induction of significant left heart structure growth and
significant myocardial recovery allowing restoration of
biventricular circulation is certainly suggested by our case
and deserves further study.
Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which per-
mits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.
References
1. Ballard G, Tibby S, Miller O, Krasemann T, Rosenthal E,
Anderson D et al (2010) Growth of left heart structures following
the hybrid procedure for borderline hypoplastic left heart. Eur J
Echocardiogr 11:870–874
2. Brown SC, Boshoff D, Eyskens B, Gewillig M (2009) Hybrid
approach as bridge to biventricular repair in a neonate with
critical aortic stenosis and borderline left ventricle. Eur J Car-
diothorac Surg 35:1080–1082
3. Eghtesady P, Michelfelder E, Altaye M, Ballard E, Hirsh R,
Beekman RH 3rd (2007) Revisiting animal models of aortic
stenosis in the early gestation fetus. Ann Thorac Surg 83:631–639
4. Emani SM, Bacha EA, McElhinney DB, Marx GR, Tworetzky
W, Pigula FA et al (2009) Primary left ventricular rehabilitation
is effective in maintaining two-ventricle physiology in the bor-
derline left heart. J Thorac Cardiovasc Surg 138:1276–1282
5. Han RK, Gurofsky RC, Lee KJ, Dipchand AI, Williams WG,
Smallhorn JF et al (2007) Outcome and growth potential of left
heart structures after neonatal intervention for aortic valve ste-
nosis. J Am Coll Cardiol 50:2406–2414
6. Makikallio K, McElhinney DB, Levine JC, Marx GR, Colan SD,
Marshall AC et al (2006) Fetal aortic valve stenosis and the
evolution of hypoplastic left heart syndrome: patient selection for
fetal intervention. Circulation 113:1401–1405
7. McCrindle BW, Blackstone EH, Williams WG, Sittiwangkul R,
Spray TL, Azakie A et al (2001) Are outcomes of surgical versus
transcatheter balloon valvotomy equivalent in neonatal critical
aortic stenosis? Circulation 104:I152–I158
8. McElhinney DB, Marshall AC, Wilkins-Haug LE, Brown DW,
Benson CB, Silva V et al (2009) Predictors of technical success
and postnatal biventricular outcome after in utero aortic valvu-
loplasty for aortic stenosis with evolving hypoplastic left heart
syndrome. Circulation 120:1482–1490
9. Salih C, Sheppard MN, Ho SY (2004) Morphometry of coronary
capillaries in hypoplastic left heart syndrome. Ann Thorac Surg
77:903–907
10. Sharland GK, Chita SK, Fagg NL, Anderson RH, Tynan M, Cook
AC et al (1991) Left ventricular dysfunction in the fetus: relation
to aortic valve anomalies and endocardial fibroelastosis. Br Heart
J 66:419–424
11. Shinkawa T, Bove EL, Hirsch JC, Devaney EJ, Hye RG (2010)
Intermediate-term results of the Ross procedure in neonates and
infants. Ann Thorac Surg 89:1827–1832
12. Smolich JJ, Walker AM, Campbell GR, Adamson TM (1989)
Left and right ventricular myocardial morphometry in fetal,
neonatal, and adult sheep. Am J Physiol 257:H1–H9
214 Pediatr Cardiol (2011) 32:211–214
123


Ross-Konno and Endocardial Fibroelastosis Resection After Hybrid Stage I Palliation in Infancy: Successful Staged Left-Ventricular Rehabilitation and Conversion to Biventricular Circulation After Fetal Diagnosis of Aortic Stenosis

Crossref

Pediatric Cardiology

Moon-Grady, Anita J.

Moore, Phillip

Azakie, Anthony

eScholarship - University of California

eScholarshipTitleRoss-Konno and Endocardial Fibroelastosis Resection After Hybrid Stage I Palliation in Infancy: Successful Staged Left-Ventricular Rehabilitation and Conversion to Biventricular Circulation After Fetal Diagnosis of Aortic StenosisPermalinkhttps://escholarship.org/uc/item/0ww1k49pJournalPediatric Cardiology, 32(2)ISSN1432-1971AuthorsMoon-Grady, Anita J.Moore, PhillipAzakie, AnthonyPublication Date2011-02-01DOI10.1007/s00246-010-9841-3 Peer reviewedeScholarship.org Powered by the California Digital LibraryUniversity of CaliforniaCASE REPORTRoss-Konno and Endocardial Fibroelastosis ResectionAfter Hybrid Stage I Palliation in Infancy: Successful StagedLeft-Ventricular Rehabilitation and Conversion to BiventricularCirculation After Fetal Diagnosis of Aortic StenosisAnita J. Moon-Grady • Phillip Moore •Anthony AzakieReceived: 24 August 2010 / Accepted: 19 September 2010 / Published online: 24 November 2010 The Author(s) 2010. This article is published with open access at Springerlink.comAbstract We report a patient who presented during fetallife with severe aortic stenosis, left-ventricular dysfunction,and endocardial fibroelastosis (evolving hypoplastic leftheart syndrome). Management involved in utero andpostnatal balloon aortic valvuloplasty for partial relief ofobstruction and early postnatal hybrid stage I palliationuntil recovery of left-ventricular systolic function hadoccurred. The infant subsequently had successful conver-sion to a biventricular circulation by combining resectionof endocardial fibroelastosis with single-stage Ross-Konno,aortic arch reconstruction, hybrid takedown, and pulmon-ary artery reconstruction.Keywords Aortic stenosis  Fetal balloon aorticvalvuloplasty  Fetal intervention  Hypoplastic left heartsyndrome  Resection of endocardial fibroelastosis Stage 1 hybridIntroductionThe morphologic and functional spectrum of the left ven-tricle in severe aortic stenosis ranges from the smaller,hypertrophied ‘‘borderline’’ left ventricle to the dilated andthin-walled left ventricle. In addition, several studies havedemonstrated that aortic stenosis with left-ventricular dys-function presenting in fetal life may lead to hypoplastic leftheart syndrome at birth, with varying degrees of chamberhypoplasia and endocardial fibroelastosis (EFE) [6, 8, 10].Neonates with severe aortic stenosis generally require eithersurgical valvotomy or transcatheter balloon valvuloplasty[7], whereas those with hypoplastic left heart are generallymanaged with univentricular palliation (Norwood or hybridtranscatheter-surgical techniques) or cardiac transplantation.Case ReportA 3.48-kg male infant was delivered by way of plannedrepeat Cesarean section at 39 2/7 weeks’ gestation to a36 year-old gravida 3, para 2 mother. The pregnancy wascomplicated by a diagnosis of fetal aortic stenosis made at22 3/7 weeks’ gestation (Fig. 1). At 23 4/7 weeks’ gesta-tion, fetal balloon aortic valvuloplasty was performed. Afterthe procedure, as demonstrated by color Doppler, aorticarch flow had gone from retrograde to antegrade across theaortic valve and in the ascending and transverse aortic arch;there was mild aortic insufficiency. Postnatally the leftventricle and mitral valve were of normal size; the aorticvalve was mildly hypoplastic at 5 mm with a peak Doppler-derived gradient of 60 mmHg; and there was significantleft-ventricular hypertrophy (diastolic left ventricular pos-terior wall dimension, LVPWd 6 mm) with poor systolicfunction (LVEF% \ 20%). Doppler flow in the transversearch and isthmus was antegrade. Cardiac catheterizationshowed increased left atrial pressure of 25 mm Hg. Theaortic valve was dilated by way of a retrograde approachwith a 5-mm Slalom balloon catheter (Cordis Corporation,Miami, FL, USA) with a decrease in peak-to-peak gradientfrom 60 to 45 mm Hg. After valvuloplasty, a 5-mm stentA. J. Moon-Grady  P. Moore  A. Azakie (&)Pediatric Heart Center, UCSF Benioff Children’s Hospital,University of California San Francisco, San Francisco,CA 94143-0117, USAe-mail: tony.azakie@ucsfmedctr.orgA. J. Moon-GradyFetal Treatment Center, UCSF Benioff Children’s Hospital,University of California San Francisco, San Francisco,CA 94143-0570, USA123Pediatr Cardiol (2011) 32:211–214DOI 10.1007/s00246-010-9841-3was placed in the interatrial septum. The neonate wasmaintained on prostaglandin infusion.On day of life 14, with no change in clinical status orLVEF%, the child underwent stage I hybrid palliation withplacement of an 18-mm ductal stent and bilateral pul-monary artery banding. Repeat dilation of the aortic valvewas performed at that time, resulting in a decrease in peak-to-peak gradient to 11 mm Hg and mild to moderate aorticinsufficiency.The LVEF% improved gradually to 60% during the sub-sequent months. Cardiac catheterization at 9 months of ageshowed a 38 mm Hg peak-to-peak gradient across the aorticvalve and mild to moderate aortic insufficiency. Left atrialpressure was 11 mm Hg; left ventricular end diastolic pressurewas 14 mm Hg; and estimated pulmonary vascular resistancewas 4 Woods units. After recovery from a severe intercurrentpulmonary illness, the child underwent a Ross-Konno pro-cedure, placement of a 16-mm right ventricle-to-pulmonaryartery homograft, ductus arteriosus and atrial stent removal,aortic arch reconstruction, pulmonary artery debanding,resection of extensive EFE (Fig. 2), and closure of an atrialseptal defect. He was successfully extubated on postoperativeday 7 and weaned from inotropic and vasodilatory support.Follow-up echocardiography showed no evidence of mitralinflow obstruction or regurgitation, normal left-ventricularsystolic function, and a well-functioning autograft with noaortic arch obstruction. At catheterization 1 month later, thecardiac index was 3.3 ml/min/M2 by thermodilution; wedgepressure mean was 10 mm Hg; and pulmonary vascularresistance was 6 Woods units (decreasing to 2.6 Woods unitsin response to inhaled nitric oxide).At 1 year of life, left heart dimensions are normal(mitral valve 13.7 mm [Z score ?0.7], LV end diastolicdiameter 24.9 mm [Z score ?0.3], and LVPWd 6 mm[Z score ?2.4]), and his systolic function is normal with anLVEF% of 69% by Simpson’s biplane method (Fig. 3).Fig. 1 Fetal echocardiogram at22 4/7 weeks’ gestation. Theleft ventricle is dilated withecho-bright endocardiumsuggestive of endocardialfibroelastosis (a), and there isleft-to-right flow across theatrial septum (b). Sagittal two-dimensional (c) and colorDoppler (d) images demonstrateretrograde perfusion of theaortic arch in red (arrowheads),and antegrade flow in the ductalarch is also apparent (blue).LA left atrium, LV left ventricle,RA right atrium, RV rightventricle212 Pediatr Cardiol (2011) 32:211–214123DiscussionExperimental models involving surgically created aorticobstruction do not recapitulate the human phenotype [3];therefore, study of the pathophysiology of this disease hasbeen difficult. However, natural history studies have shownthat in the presence of certain physiologic findings (spe-cifically left-to-right atrial shunting and retrograde aorticarch perfusion in fetal life), progression to hypoplastic leftheart syndrome at term can be reliably predicted [6]. Therationale for fetal aortic valvuloplasty is to relieve aorticobstruction and promote antegrade flow through the leftheart and aorta. With restoration of antegrade flow, weobserved continued growth of the left heart structuresthrough the remainder of gestation; however, ventricularfunction did not improve appreciably. The reason for this isspeculative, but it has been proposed that the fetal leftventricle responds to obstruction with increase in masswithout increase in capillary density, which may result in ahypertrophied myocardium with increased susceptibility toischemia [9, 12]. The extensive EFE in this patient as wellas the observed hypertrophy might support this theory. Fetalvalvuloplasty partially relieved the obstruction, and afterbirth we undertook a combined approach that may havefurther mitigated the effect of long-standing obstruction andischemic insult through relief of obstruction (by way ofadditional valvuloplasties) and decrease in preload (leftatrial decompression with atrial stenting). Facilitatingantegrade perfusion of the aorta and coronary circulationwith normally saturated blood, along with improvement inmyocardial perfusion, may have contributed to the slowimprovement in the patient’s systolic and diastolic function.Previous reports have also documented the possibility ofreversible severe left-ventricular dysfunction and ventricu-lar growth in the neonate [1, 2, 5]. Emani et al. [4] reported aseries of patients with borderline left heart disease whounderwent ‘‘primary left-ventricular rehabilitation’’ at amedian age of 5.6 months, with aortic and/or mitral valvesurgery and EFE resection, with good short-term results andno deaths at a median follow-up of 25 months. Our case,however, represents the first report to our knowledge of apatient initially palliated with a hybrid transcatheter-surgi-cal approach after fetal intervention followed by successfulsurgical takedown of the hybrid and conversion to biven-tricular circulation by adding Ross-Konno procedure andaortic arch reconstruction to the EFE resection.Fig. 2 Surgical specimens ofthe explanted aortic valve (inset,left) and left-ventricularendocardium. The aortic valvewas severely dysplastic,doming, and unicuspid. At thetime of biventricular repair, thevalve was detached from theaortic wall. The endocardiumwas diffusely thickened andexcised from base to apexthrough the left-ventricularoutflow tractFig. 3 Echocardiogram 2 months after biventricular repair demon-strates an apex-forming left ventricle and mild residual left-ventric-ular hypertrophyPediatr Cardiol (2011) 32:211–214 213123Long-term outcome for patients undergoing extensiveEFE resection as part of left-ventricular rehabilitation areunknown. Medium-term results from patients undergoingneonatal Ross-Konno procedures have been mixed, andparticularly poor results in patients requiring concomitantaortic arch reconstruction have been noted. However, arecent large single-center experience [11] suggested thatoverall survival and functional status may representimprovement compared with single-ventricle palliation orneonatal transplantation. Certainly long-term follow-updata, including evaluation of functional and neurodevel-opmental status and comorbidities (including pulmonaryvascular disease) will be needed before there is widespreadadoption of this type of approach. However, feasibility ofinduction of significant left heart structure growth andsignificant myocardial recovery allowing restoration ofbiventricular circulation is certainly suggested by our caseand deserves further study.Open Access This article is distributed under the terms of theCreative Commons Attribution Noncommercial License which per-mits any noncommercial use, distribution, and reproduction in anymedium, provided the original author(s) and source are credited.References1. Ballard G, Tibby S, Miller O, Krasemann T, Rosenthal E,Anderson D et al (2010) Growth of left heart structures followingthe hybrid procedure for borderline hypoplastic left heart. Eur JEchocardiogr 11:870–8742. Brown SC, Boshoff D, Eyskens B, Gewillig M (2009) Hybridapproach as bridge to biventricular repair in a neonate withcritical aortic stenosis and borderline left ventricle. Eur J Car-diothorac Surg 35:1080–10823. Eghtesady P, Michelfelder E, Altaye M, Ballard E, Hirsh R,Beekman RH 3rd (2007) Revisiting animal models of aorticstenosis in the early gestation fetus. Ann Thorac Surg 83:631–6394. Emani SM, Bacha EA, McElhinney DB, Marx GR, TworetzkyW, Pigula FA et al (2009) Primary left ventricular rehabilitationis effective in maintaining two-ventricle physiology in the bor-derline left heart. J Thorac Cardiovasc Surg 138:1276–12825. Han RK, Gurofsky RC, Lee KJ, Dipchand AI, Williams WG,Smallhorn JF et al (2007) Outcome and growth potential of leftheart structures after neonatal intervention for aortic valve ste-nosis. J Am Coll Cardiol 50:2406–24146. Makikallio K, McElhinney DB, Levine JC, Marx GR, Colan SD,Marshall AC et al (2006) Fetal aortic valve stenosis and theevolution of hypoplastic left heart syndrome: patient selection forfetal intervention. Circulation 113:1401–14057. McCrindle BW, Blackstone EH, Williams WG, Sittiwangkul R,Spray TL, Azakie A et al (2001) Are outcomes of surgical versustranscatheter balloon valvotomy equivalent in neonatal criticalaortic stenosis? Circulation 104:I152–I1588. McElhinney DB, Marshall AC, Wilkins-Haug LE, Brown DW,Benson CB, Silva V et al (2009) Predictors of technical successand postnatal biventricular outcome after in utero aortic valvu-loplasty for aortic stenosis with evolving hypoplastic left heartsyndrome. Circulation 120:1482–14909. Salih C, Sheppard MN, Ho SY (2004) Morphometry of coronarycapillaries in hypoplastic left heart syndrome. Ann Thorac Surg77:903–90710. Sharland GK, Chita SK, Fagg NL, Anderson RH, Tynan M, CookAC et al (1991) Left ventricular dysfunction in the fetus: relationto aortic valve anomalies and endocardial fibroelastosis. Br HeartJ 66:419–42411. Shinkawa T, Bove EL, Hirsch JC, Devaney EJ, Hye RG (2010)Intermediate-term results of the Ross procedure in neonates andinfants. Ann Thorac Surg 89:1827–183212. Smolich JJ, Walker AM, Campbell GR, Adamson TM (1989)Left and right ventricular myocardial morphometry in fetal,neonatal, and adult sheep. Am J Physiol 257:H1–H9214 Pediatr Cardiol (2011) 32:211–214123

AC et al (2006) Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention.

Azakie A et al (2001) Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis? Circulation 104:I152–I158

Beekman RH 3rd (2007) Revisiting animal models of aortic stenosis in the early gestation fetus. Ann Thorac Surg 83:631–639

Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart.

Hybrid approach as bridge to biventricular repair in a neonate with critical aortic stenosis and borderline left ventricle.

Intermediate-term results of the Ross procedure in neonates and infants. Ann Thorac Surg 89:1827–1832

Left and right ventricular myocardial morphometry in fetal, neonatal, and adult sheep.

Left ventricular dysfunction in the fetus: relation to aortic valve anomalies and endocardial ﬁbroelastosis.

Morphometry of coronary capillaries in hypoplastic left heart syndrome. Ann Thorac Surg 77:903–907

Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis.

Pigula FA et al (2009) Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart.

Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome.

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Ross-Konno and Endocardial Fibroelastosis Resection After Hybrid Stage I Palliation in Infancy: Successful Staged Left-Ventricular Rehabilitation and Conversion to Biventricular Circulation After Fetal Diagnosis of Aortic Stenosis

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