A combined stage 1 and 2 repair for hypoplastic left heart syndrome: anaesthetic considerations

Case report

A combined stage 1 and 2 repair for hypoplastic leftheart syndrome: anaesthetic considerations

MATTHIAS MULLER M DM D*, HAKAN AKINTURK M DM D†,

EHRENFRIED SCHINDLER M DM D*, MICHAEL BRAU M DM D*,

STEFAN SCHOLZ M DM D*, KLAUS VALESKE M DM D†, INA

MICHEL-BEHNKE M DM D‡, JOSEF THUL M DM D‡,6 DIETMAR

SCHRANZ M DM D‡ AND GUNTER HEMPELMANN M DM D*

Departments of *Anaesthesiology, Intensive Care, Pain Therapy, †Cardiac and Pediatric CardiacSurgery and ‡Pediatric Cardiology, University Hospital Giessen, Germany

SummaryTherapy of hypoplastic left heart syndrome (HLHS) consists of the

staged Norwood procedure or cardiac transplantation. Stenting the

ductus arteriosus and subsequent banding of the pulmonary arteries

allows the combination of neoaortic reconstruction with the estab-

lishment of a bidirectional cavopulmonary connection (combined

stage 1 and 2 procedure) in a later session. We report the anaesthetic

management in eight infants ranging from 107 to 195 days undergoing

a combined stage 1 and 2 procedure. Nonselective pulmonary

vasodilators and nitric oxide were needed in all cases to improve

oxygen saturation in the postbypass period. Phosphodiesterase inhib-

itors and epinephrine were required in all patients for inotropic

support during and after weaning off cardiopulmonary bypass. The

procedure was successful in seven patients. One patient died intra-

operatively because of right heart failure. The physiological changes of

this new surgical strategy for palliation of HLHS offers a challenge for

the anaesthetist primarily in the early postbypass period.

Keywords: hypoplastic left heart syndrome; cardiac surgery;

Norwood procedure; anaesthesia

Introduction

Therapy of hypoplastic left heart syndrome (HLHS)

consists of the staged Norwood procedure or cardiac

transplantation (HTX2 ). Outcome of patients planned

for HTX primarily depends on the availability of a

donor heart. In our institution the mean waiting time

for infants is 50 days for a donor heart (1), therefore

many infants die while waiting for an organ. The

outcome of the Norwood procedure is mainly

influenced by a successful first stage operation (2).

A significant number of patients die, however,

before the second stage of the Norwood procedure

can be performed (3,4). Stenting the ductus arterio-

sus in combination with pulmonary artery banding

and atrial septostomy offers a new approach to

Correspondence to: Dr Matthias Muller, Department of Anaes-thesiology, Intensive Care, Pain Therapy, University HospitalGiessen, Rudolf-Buchheim-Str. 7, 35392 Giessen, Germany (email:[email protected]).

Paediatric Anaesthesia 2003 13: 360–365

360 � 2003 Blackwell Publishing Ltd

palliation of HLHS (5). Neoaortic reconstruction and

establishment of a bidirectional cavopulmonary

connection (BCPC) can then be performed during a

single operation (combined stage 1 and 2 proce-

dure). The technical details have been described

recently (6).

The anaesthetic management in patients under-

going a combined stage 1 and 2 procedure for HLHS

has not been described. In this report we review our

experiences in eight infants undergoing this novel

surgical technique from the anaesthetic point of

view.

Methods and results

The charts of eight patients with different forms of

HLHS (Table 1) undergoing a combined stage 1 and

2 procedure were reviewed retrospectively. At the

time of the combined stage 1 and 2 procedure the

median age of the patients was 151 days, ranging

from 107 to 195 days (Table 1).

Preoperative cardiac catheterizationand pulmonary artery banding

Our patients were having conventional intravenous

prostaglandin E1 therapy until a ‘Peripheral Jo-stent’

(Jomed, Ramendingen, Germany) or a ‘Saxx-stent’

(Devon, Hamburg, Germany) with a length of 12, 17

or 20 mm was placed in the ductus arteriosus.

Additionally, gradual balloon dilation atrial septos-

tomy was performed in patients who did not have

unrestricted blood flow through a large atrial septal

defect. Balloon catheters with final diameters of 10

and 12 mm were used. One to three days following

this interventional procedure bilateral pulmonary

artery banding was performed to reduce systolic

pulmonary artery pressure to less than half systolic

systemic arterial pressure and to decrease arterial

oxygen saturation in room air to 80 ± 5%. Before

discharge 8–21 days after pulmonary artery banding

haemodynamics were reevaluated by further cardiac

catheterization. Reevaluation of the clinical course,

echocardiography and electrocardiography was

performed every 1–2 weeks on an outpatient basis.

To plan the combined stage 1 and 2 procedure, a

preoperative cardiac catheterization was routinely

performed 3–5 months after pulmonary artery band-

ing. The courses of pulmonary artery pressure,

cardiac filling pressures and oxygen saturation are

presented in Table 2.

Anaesthesia and monitoring

All infants were unpremedicated and had a small

intravenous cannula in situ. Anaesthesia was

induced with fentanyl 15 lgÆkg)1 and pancuronium

0.4 mgÆkg)1. The lungs were ventilated with an FiO2

of 0.21–0.5 to maintain an SpO2 of approximately

85%. After arterial cannulation and nasotracheal

intubation, a five French double-lumen central

venous catheter was inserted via the jugular or

subclavian vein into the superior vena cava and

another catheter was placed via the femoral vein into

the inferior vena cava. Besides measuring arterial,

central venous and pulmonary artery pressure (after

construction of the BCPC via the vena caval superior

catheter), further perioperative monitoring consisted

of pulse oximetry, endtidal CO2, temperature (oeso-

phageal and rectal), urine output and blood gas

Table 1

Biometric data, diagnosis,preanaesthetic drug treatmentand outcome

Treatment beforeanaesthesia

Patientnumber Diagnosis

Age(days)

Weight(kg)

Height(cm) Ventilated Drugs Outcome

1 MS/AS 142 5.6 64 ) Pentobarbital Alive2 MA/AA 117 4.2 61 ) Catecholamines Dead3 MS/AS 178 5.2 59 ) – Alive4 MS/AA 107 5.5 61 ) – Alive5 MS/AS 117 4.8 58 ) – Alive6 L-TGA/AA 195 5.3 65 + – Alive7 MS/AS 159 5.8 66 ) – Alive8 MS/AS 180 5.7 70 ) – Alive

MS, mitral stenosis; AS, aortic stenosis; MA, mitral atresia; AA, aortic atresia; L-TGA, correctedtransposition of the great arteries.

COMBINED STAGE 1 AND 2 REPAIR FOR HLHS 361

� 2003 Blackwell Publishing Ltd, Paediatric Anaesthesia, 13, 360–365

analyses. Anaesthesia was maintained with fentanyl

25–50 lg every 45–60 min (total dose ranging from

150 to 300 lgÆkg)1) and midazolam (0.2 mgÆkg)1)

after starting cardiopulmonary bypass (CPB) and

repeated when necessary before weaning from CPB

(total dose ranging from 0.7 to 2.7 mgÆkg)1). Before

deep hypothermic circulatory arrest (DHCA) all

infants received a repeat dose of pancuronium,

pentobarbital 100 mg and dexamethasone 1 mgÆkg)1

for cerebral protection.

Cardiopulmonary bypass

The median duration of CPB and DHCA was

322 min (ranging from 279 to 440 min) and 75 min

(ranging from 63 to 90 min), respectively.

The CPB was performed using nonpulsatile per-

fusion (2.4 lÆmin)1m)2) with a membrane oxygenator

(Lilliput 2; Dideco, Torino, Italy). Priming of the

extracorporeal circuit consisted of 325 ml Ringer’s

solution, 150 ml 20% albumin solution and 35 ml of

8.4% bicarbonate. One unit of packed red cells

(300 ml) and mannitol (0.45 gÆkg)1) were added to

the circuit before onset of CPB. No steroids were

added to the priming solution and no vasodilators

were given during CPB. A haemofilter was incor-

porated into the CPB circuit to concentrate blood in

the CPB equipment without loss of plasma fraction

whenever possible (7). Prior to the onset of CPB, a

loading dose of heparin (300 UÆkg)1) was adminis-

tered for anticoagulation followed by a 50% loading

dose every hour. The arterial cannula was placed in

the pulmonary trunk and for venous drainage a

bicaval cannulation technique was used. During

hypothermic CPB perfusion, flow rate was reduced

to achieve a continuously measured venous SvO2

>70%. As DHCA was used in all cases , the infants

were cooled down to a rectal temperature of 18�C.

Additionally ice-packs were placed around the head

for topical cooling. During cooling and rewarming, a

maximum temperature gradient of 10�C between

patients rectal temperature and perfusate was

accepted. The acid–base management followed the

a-stat methodology. During CPB the packed cell

Table 2

Haemodynamic parameters and oxygen saturation from preoperative cardiac catheterizations and postoperatively before discharge toPICU

At admission After PA banding Before stage 1 and 2 After stage 1and 2

Median Range Median Range Median Range Median Range

SpO2 (%) 90 78–97 80 75–85 78 76–91 61 51–71MAP (mmHg) 53 40–64 57 47–71 64 45–79 51 46–57MPAP (mmHg) 50 45–58 14a 10–19 13a 12–25 18 12–25dRVP (mmHg) 11 5–11 9 3–15 9 3–12 11b 8–16

PA banding, pulmonary artery banding; SpO2, arterial oxygen saturation; MAP, mean arterial pressure; MPAP, mean pulmonary arterypressure; dRVP, diastolic right ventricular pressure; ameasurement distal to the PA banding; bcentral venous pressure.

Table 37

Drug therapy during and afterweaning off bypass

Patient number 1 2 3 4 5 6 7 8

CatecholaminesDobutamine (5–10 lgÆkg)1Æmin)1) ) + + + + + ) )Epinephrine (0.1–1.5 lgÆkg)1Æmin)1) + + + + + + + +Norepinephrine (0.1–1.0 lgÆkg)1Æmin)1) + ) ) ) ) ) ) )

PDE-III inhibitorsMilrinone (1.0 lgÆkg)1Æmin)1) ) ) ) + + + + +Enoximone (10 lgÆkg)1Æmin)1) + + + ) ) ) ) )

VasodilatorsNitroglycerine (5 lgÆkg)1Æmin)1) + + + + + + + +Nitroprusside (0.5–10 lgÆkg)1Æmin)1) ) + ) + ) ) ) )Prostaglandin E1 (0.05–0.25 lgÆkg)1Æmin)1) + + ) + + + + )NO (15–20 p.p.m.) + + + + + + + +

PDE, phosphodiesterase inhibitor; NO, nitric oxide.

362 M. MULLER ET AL.


volume was maintained (depending on patient

temperature) between 20 and 30%. Weaning off

CPB was started when the rectal temperature

reached 36�C. After successful weaning from CPB,

protamine was given in a 1 : 1 ratio to the total

amount of heparin. Blood products (packed red

cells, fresh frozen plasma and platelets) were given

as necessary in the postbypass period.

Drugs

One patient received catecholamines preoperatively

(Table 1). This patient could not be weaned success-

fully from CPB and died because of refractory right

heart failure.

Drug therapy during weaning from CPB and in

the postbypass period is summarized in Table 3.

Routinely, nitroglycerine and a phosphodiesterase

(PDE)-III inhibitor were started before weaning from

CPB. The contractility of the heart was assessed by

direct visualization and dobutamine was given

when necessary, if the heart rate was <170 bÆmin)1.

Adrenaline was used as third-line inotropic drug.

Vasopressor support was indicated, when mean

arterial pressure (MAP) was lower than 45 mmHg

despite optimal preload (3 CVP 8–12 mmHg). Norad-

renaline was used in one infant and nitroprusside in

two infants to reduce ventricular afterload. To

decrease pulmonary vascular resistance and to

improve oxygenation, all patients received intraven-

ous nitroglycerine, additionally prostaglandin E1

and thereafter inhaled nitric oxide (iNO) were used

when oxygen saturation was less than 70%.

Alkalization was performed and 100% oxygen

was given during weaning from CPB. The course of

SpO2 after successful weaning from CPB is demon-

strated in Figure 1. Inhaled NO increased signifi-

cantly median SpO2 from 48% (range 40–63%) to

61% (range 51–71%, P ¼ 0.015, Wilcoxon matched

pairs test). However, a trend towards a lower mean

pulmonary artery pressure could be observed in

response to iNO (median, 20 mmHg; range 12–

32 mmHg vs median, 18 mmHg; range 12–

25 mmHg, P ¼ 0.687, Wilcoxon matched pairs test).

During the postbypass period the median haemo-

globin was 8.8 gÆdl)1 (range 8.1–10.3 gÆdl)1) and

median pH was 7.46 (range 7.39–7.56). The median

diuresis in this period was 14.5 mlÆh)1 (range 1.4–

32.6 mlÆh)1).

Postoperative care

All infants apart from infant no. 2, who died in the

OR4 , were discharged to the paediatric intensive care

unit (PICU). During the postoperative stay an

interventional stent placement was necessary in

three patients because of stenosis of the left pul-

monary artery or coarctation of the aorta. The

diagnosis was first made by Doppler echocardiog-

raphy and than verified by cardiac catheterization.

The patients were ventilated for 7–11 days (median,

8 days). The total duration of PICU and hospital stay

ranged between 10 and 22 days (median, 16 days),

and between 21 and 59 days (median, 24 days),

respectively. At the time of discharge from the

paediatric department the median SpO2 of the

remaining seven patients was 81% (range 76–83%).

Discussion

The most critical period of the procedure is the

weaning from CPB and early postbypass period. It

has been reported that an increased pulmonary

vascular resistance (PVR), either directly or as a result

of the systemic inflammatory response after CPB,

has a significant effect on postoperative recovery of

infants after cardiac surgery (8). Impaired endothe-

lium-dependent vasodilatation is present in children

with high pulmonary flow and pressure (9) which

might be exacerbated by CPB (10). As the effective

pulmonary flow after BCPC depends on a low PVR,

decreasing PVR is a main target in the postoperative

management of these patients. It has been shown in

Inhaled nitric oxide (iNO)

Without iNO With iNO0

10

20

30

40

50

60

70

80

90

Oxy

gen

satu

ratio

n (%

)

Figure 1

Individual postcardiopulmonary bypass course of oxygen satura-tion in the seven survivors.



patients undergoing Fontan-type operations that

even minor increases in PVR can result in severe

impairment of pulmonary perfusion (11). Therefore

nonselective pulmonary vasodilators including

nitroglycerine and prostaglandin were infused in all

patients to decrease pulmonary vascular resistance.

Furthermore iNO was added in all patients to

improve arterial oxygen saturation after weaning

from CPB. As we observed only a trend towards

lower mean pulmonary artery pressures after iNO,

we speculate that an improved ventilation–perfusion

matching may additionally explain the increase in

oxygen saturation after iNO. Improved oxygenation

after iNO therapy has been reported in children and

adults with acute respiratory failure without pul-

monary hypertension (12,13). However, an increase

in oxygen saturation together with a decrease in

pulmonary artery pressure after iNO in children

with BCPC has been reported by other groups (14,15).

Despite its acceptance as an effective palliative

procedure, there is reluctance to perform a BCPC in

very young infants because of an elevated or labile

pulmonary vascular resistance. The influence of age

on the outcome of elective BCPC as a primary or

secondary palliative procedure has been analysed by

Reddy et al. They found no difference in outcome in

infants <6 months compared with older infants or

children (16). In our patients a low mean pulmonary

artery pressure distal to the banding preoperatively,

together with an adequate oxygen saturation, and no

echocardiographic signs of excessive pressure or

volume load on the systemic ventricle indicates an

effective pulmonary artery banding as a good basis

for the BCPC.

In contrast with a standard BCPC which can be

performed without circulatory arrest, the combined

stage 1 and 2 procedure leads to ventricular dys-

function because of myocardial ischaemia and rep-

erfusion. Inotropic support was necessary in all

patients, and (PDE)-III inhibitors (enoximone or

milrinone) were used as first-line agents because of

their pulmonary vasodilating and inotropic effects

(17,18). Catecholamines were required, although they

can be associated with a deterioration of the diastolic

ventricular function (19). Moreover, epinephrine and

norepinephrine possess vasoconstrictive effects,

which are not favourable in a BCPC physiology.

Using this inotropic support we found no evidence of

a reduced cardiac output in the survivors with

respect to acidosis and diuresis. The large dose range

for epinephrine and norepinephrine results mainly

from the patient who could not be weaned from CPB

despite high doses of inotropic and vasopressor

support. This infant developed a low cardiac output

preoperatively at home, because of stenosis of the

aortic part of the ductus arteriosus. Right heart failure

persisted despite emergency balloon dilatation. The

parents refused HTX, so reconstructive surgery was

performed as a rescue approach.

Coarctation of the aorta and an extrinsic pulmon-

ary artery stenosis were seen in three of the first four

patients. Both major complications are strongly

associated with the surgical procedure. The initial

surgical technique, placement of an allograft tube

from the proximal divided main pulmonary artery

to the arch and proximal descending aorta, was

abandoned in the subsequent patients (6). No patient

has yet come to completion of the Fontan procedure.

Although this last step to Fontan completion is

associated with low mortality (20), these data are

necessary to assess the overall mortality and mor-

bidity of our strategy for the treatment of HLHS.

In conclusion, we present the perioperative man-

agement for palliation of infants with HLHS with a

stent in the ductus arteriosus and pulmonary artery

banding with subsequent combined stage 1 and 2

surgical repair. This strategy may be an alternative

to the conventional treatment options in patients

with HLHS. However, more experience is necessary

to optimize the anaesthetic and intensive care

management of this particular patient population.

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Accepted 20 November 2002



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A combined stage 1 and 2 repair for hypoplastic left heart syndrome: anaesthetic considerations