Download Fontan Operation in a Patient with Severe Hypoplastic Right

Acta Cardiol Sin 2016;32:612-615
Case Report
doi: 10.6515/ACS20160105A
Fontan Operation in a Patient with Severe
Hypoplastic Right Pulmonary Artery, Single
Ventricle, and Heterotaxy Syndrome
Jun-Yen Pan,1 Chu-Chuan Lin2 and Jen-Ping Chang3
Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical
distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary
arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances,
some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For
theses patients, completion of a Fontan operation will be challenging. We reported a 17-year-old girl with a single
ventricle and heterotaxy syndrome and only her left lung functioning, who underwent one-lung Fontan operation
with a satisfactory result.
Key Words:
Anomaly · Cardiovascular surgery · Congenital heart disease
INTRODUCTION
CASE REPORT
Preoperative assessment of a patient’s pulmonary
artery circulation including pulmonary pressure, pulmonary vascular resistance, pulmonary artery size, and absence of pulmonary distortion is important before undergoing a Fontan operation.1 However, in rare instances,
some patients have just a single ventricle, with only one
functioning lung. Completion of a one-lung Fontan operation is one of the most challenging surgical situations.2
We reported a 17-year-old girl with a single ventricle and
heterotaxy syndrome and only a functioning left lung,
who successfully underwent one-lung Fontan operation.
This female patient was born in September 1992
with a diagnosis of heterotaxy syndrome with situs inversus, dextrocardia, right atrial isomerism, common
atrium, single atrioventricular valve, single morphological right ventricle, pulmonary atresia, right pulmonary
artery hypoplasia, and bilateral superior vena cava (Figure 1A). She was referred to our hospital at 5 years of
age with a functioning left modified Blalock-Taussig
shunt which had been created during the child’s neonate period in another hospital. Her cardiac catheterization showed an absence of main and right pulmonary
arteries, patent previous left Blalock-Taussig shunt and
left pulmonary artery. Exploratory right thoracotomy
was performed at that time and severe right pulmonary
artery hypoplasia (1 mm in diameter) was identified.
She had been regularly followed since then.
Subsequently, the patient underwent bilateral superior venae cavae to the left pulmonary artery connection and left Blalock-Taussig shunt division at 11 years of
age due to progressive cyanosis (Figure 1B, C). The postoperative systemic oxygen saturation was around 85% in
room air. At 17 years of age, she was again medically
Received: June 11, 2015
Accepted: January 5, 2016
1
Department of Cardiovascular Surgery; 2Department of Pediatrics,
Kaohsiung Veterans General Hospital; 3Department of Thoracic and
Cardiovascular Surgery, Kaohsiung Chang Gung Memorial Hospital,
Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Address correspondence and reprint requests to: Dr. Jen-Ping Chang,
Division of Thoracic and Cardiovascular Surgery, Kaohsiung Chang
Gung Memorial Hospital; Chang Gung University College of Medicine,
No. 123, Tapei Rd., Niaosung District, Kaohsiung City 83301, Taiwan.
Tel: 886-7-731-7123 ext. 8008; Fax: 886-7-731-8762; E-mail: c9112772
@adm.cgmh.org.tw
Acta Cardiol Sin 2016;32:612-615
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One-Lung Fontan
A
B
C
Figure 1. (A) The preoperative 3-dimensional computed tomographic angiography demonstrated the gross anatomy of this single ventricle with
heterotaxy syndrome. The diminutive right pulmonary arteries were also demonstrated. (B) A pulmonary angiography performed before Fontan completion demonstrated the patent bilateral superior venae cavae to left pulmonary artery connection with good size and flow. (C) A drawing demonstrated the anatomical orientation after bilateral superior venae cavae to left pulmonary artery connection. CA, common atrium; IVC, inferior vena
cava; L, left superior vena cava to left pulmonary artery connection; LPA, left pulmonary artery; R, right superior vena cava to left pulmonary artery
connection; RPA, right pulmonary artery; SRV, single right ventricle.
scrutinized because of recurrent cyanosis (systemic oxygen saturation was around 70%) and dyspnea on exertion. The cardiac catheterization showed the ventricular
end-diastolic pressure of 12 mmHg with moderate atrioventricular valve regurgitation. The mean pulmonary artery pressure was 14 mmHg, and the inferior vena cava
pressure was 16 mmHg. There were some systemic-topulmonary collateral vessels to the right upper lung, but
no major aorto-pulmonary collateral artery was noted.
The calculated pulmonary artery index (PAI) was 82.8
mm2/m2, pulmonary vascular resistance was 2.43 WU.m2,
and the McGoon ratio was 0.63. Although, the smaller
PAI and moderate atrioventricular valve regurgitation
were thought to be the risk factors for the Fontan completion, the low pulmonary artery pressure of 14 mmHg,
acceptable pulmonary vascular resistance of 2.43 WU.m2,
and fairly lower systemic oxygen saturation of only 70%
suggested that the pulmonary system may have some
capacity for more venous blood to pass through in this
particular patient. Furthermore, the ventricular end-diastolic pressure might be reduced if the atrioventricular
valve regurgitation could be improved by either repair
or replacement. In 2009, under normothermic cardiopulmonary bypass without cardioplegic arrest, extracardiac conduit total cavopulmonary connection with 20
mm Gore-Tex Stretch Vascular Graft (W. L. Gore & Assoc,
Flagstaff, AZ, USA) was performed between the inferior
vena cava and the left pulmonary artery. A fenestration
with 6 mm Gore-Tex vascular graft interposition between the conduit and the common atrium was created
concomitantly (Figure 2). Subsequently, under cardioplegic arrest, the atrioventricular valve regurgitation
due to an anterior leaflet cleft and annular dilation was
Figure 2. The computed tomographic angiography after Fontan completion showed the appearance of left sided extracardiac conduit total
cavopulmonary connection and the intracardiac anatomy. The situs
inversus was also demonstrated. CA, common atrium; EC, extracardiac
conduit; L, left superior vena cava to left pulmonary artery connection;
SAVV, single atrioventricular valve; SRV, single right ventricle.
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Jun-Yen Pan et al.
darone 200 mg per day, and furosemide 20 mg per day.
repaired with cleft closure using interrupted stitches
and commissural suture annuloplasty with Reed’s technique. The cardiopulmonary bypass time and the cardiac ischemic time was 240 minutes and 46 minutes, respectively.
The patient’s postoperative course was uneventful.
The endotracheal tube was removed 12 hours postoperatively and the drain tubes were removed 6 days after
the operation. Postoperative pulmonary pressure was
20 mmHg and the systemic oxygen saturation was 94%
on room air. The postoperative two-dimensional echocardiography confirmed trivial atrioventricular regurgitation. She was discharged 20 days after the operation,
remaining until the left pleural effusion was resolved.
One episode of paroxysmal superior ventricular tachycardia was noted and that was controlled with amiodarone beginning 6 months after the operation. The follow-up cardiac catheterization one year after the operation showed the ventricular end-diastolic pressure of 11
mmHg and the pulmonary artery pressure of 20 mmHg.
The systemic oxygen saturation was 91% on room air. A
percutaneous transcatheter pulmonary arteriovenous
fistula embolization for the repetitive right upper pulmonary hemorrhage manifested as hemoptysis without
any compromise of the oxygen saturation, was performed at the second postoperative year. Otherwise,
during the 5-year follow-up, she experienced a fairly
normal life style with mild fatigue on exertion (New York
Heart Association functional class I to II) under a regimen of dipyridamole 75 mg three times per day, amio-
DISCUSSION
The first case of one-lung Fontan operation was reported by Sade et al.3 According to the report by Fujii et
al. in 2012, 2 the total number of reported one-lung
Fontan operations including this case will be 29 (Table
1).2-8 In that report, they found that the impaired ventricular function was a significant risk factor for mortality by univariate analysis. Furthermore, a higher incidence of 13.8% (4 out of 29) of protein-losing enteropathy and a higher overall mortality rate of 20.7% (6 out
of 29) were identified in this particular group of patients
(Table 1).
In cases of irreparable congenital or acquired atresia
of one or the other branch pulmonary artery, clinicians
are left to contemplate the possibility of Fontan operation to one lung. The complete loss of a pulmonary artery means an almost 50% reduction of PAI, which has
been shown to have a significant detrimental effect to
the pulmonary vascular compliance in Fontan patients.1
A PAI less than 100 mm2/m2 typically results in higher
central venous pressure and increased afterload to the
single ventricle after the Fontan operation. Knott-Craig
et al. reported that patients with early failure or persistent effusions had a lower PAI than those with good outcome (185 ± 17 versus 276 ± 83 mm2/m2, p < 0.01) after
a modified Fontan operation for 30 patients with tri-
Table 1. Demographics of the reported one-lung Fontan operation
N
3
Sade 1989
1
4
Subbareddy 1996 1
5
7
Zachary 1998
Jacobs 2004
6
Al-Khaldi 2005 7
8
Pereira 2009
2
Fujii 2012
5
1
1
12
Diagnosis
Functioning lung
Result
Completion
Late complication
DILV
TA
HLHS: 5; DORV:1;
SV: 1
HLHS: 4; TA: 1
Right
Right
Right: 7
Alive
Alive
Late death: 2
Fontan (valved)
Fontan
TCPC: 7 (2 fenestrated)
Right: 5
Alive
SSS
None
Pleural effusion:4
(3 fenestration)
PLE: 3
SV
Ebstein
DORV: 4; SV: 4;
TA: 2; HLHS: 1;
PAIVS: 1
Left
Right
Right: 9; Left: 3
TCPC: 4 (3 fenestrated);
Lateral Fontan: 1
Alive
TCPC (fenestrated)
Alive
Fontan (fenestrated)
In-hospital death: 1; TCPC: 11 (7 fenestrated);
Late death: 2
Fontan: 1
PSVT
Hemoptysis
Bronchitis: 1;
Hemoptysis: 1;
PLE: 1; PSVT: 1
DILV, double inlet left ventricle; DORV, double outlet right ventricle; HLHS, hypoplastic left heart syndrome; PAIVS, pulmonary
atresia with intact ventricular septum; PLE, protein-losing enteropathy; PSVT, paroxysmal supraventricular tachycardia; SSS, sick
sinus syndrome; SV, single ventricle; TA, tricuspid atresia; TCPC, total cavopulmonary connection.
Acta Cardiol Sin 2016;32:612-615
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One-Lung Fontan
cuspid atresia.9 In contrast, Bridges et al. showed that
there was no statistical difference in PAI between the
survivors and the non-survivors in 29 patients who underwent modified Fontan operation.10 In their study, the
lowest PAI associated with survival was 48 mm2/m2.
Considering the donor shortage and the suboptimal
long-term survival of recent pediatric heart transplantation, we agree with the perspective addressed by Fujii et
al., that we must not exclude patients as candidates for
the Fontan operation solely because of the marginal
PAI.2 Through careful preoperative evaluation and planning, it is possible to perform the one-lung Fontan operation for the patients with a single ventricle and a single
lung. Although the postoperative pulmonary artery pressure will be high (20 mmHg in our patient) and the related long-term complications will be an anticipated issues, absence of one lung should not, in itself, be a contraindication to the Fontan operation.
2.
3.
4.
5.
6.
7.
8.
CONFLICT OF INTERESTS
9.
None declared.
10.
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