Biomedical subjects

Lennart F Duebener

Publications and source records attributed to Lennart F Duebener.

12 recordsLinked to original sources

PubMed2005

Ross procedure and left ventricular mass regression.

BACKGROUND: Return of left ventricular mass to normal is considered to be a favorable result of aortic valve replacement. The Ross procedure provides near normal hemodynamics and thus allows studies of left ventricular (LV) reverse remodeling. LV mass regression may be influenced by surgical technique (subcoronary [SC] versus root replacement [RR]). METHODS AND RESULTS: Data from the German Ross Registry were analyzed. A total of 646 patients (mean age: 43.6+/-12.7 years, range: 16 to 71 years; SC technique n=295, RR technique n=351) underwent a Ross procedure in 7 participating centers from 1990 to 2004. The patients underwent preoperative and postoperative echocardiographic evaluations. Mean follow-up time was 3.5+/-2.5 years (range 0.12 to 13.7 years). Follow-up completeness was 97%. The LV mass index (LVMI) decreased significantly during follow-up in both groups (SC: 209+/-53 preoperatively to 154+/-48 at 1-year follow-up, [P<0.01 versus preoperative values] to 149+/-51 g/m2 at 2-year follow-up, [P=NS 1-year versus 2-year follow-up] versus RR: from 195+/-56 preoperatively to 144+/-51 at 1-year follow-up [P<0.01 versus preoperative values] to 140+/-49 g/m2 [P=NS 1-year versus 2-year follow-up]). LVMI regression remained stagnant 1 year after the Ross procedure in most patients in both groups. On the basis of multivariate analysis, predictors for incomplete LVMI regression after the autograft procedure were high preoperative LVMI, smoking, and uncontrolled diastolic hypertension. CONCLUSIONS: At mid-term echocardiographic follow-up, patients of both groups had favorable autograft hemodynamics. Risk factors for incomplete postoperative LVMI regression in our study were smoking and persistent diastolic hypertension. This emphasizes the importance of cessation of smoking and treatment of arterial hypertension, even in younger patients, after corrected aortic valve disease.

Adolescent↗

PubMed2005

Tissue oxygenation index is a useful monitor of histologic and neurologic outcome after cardiopulmonary bypass in piglets.

OBJECTIVE: Tissue oxygenation index is a novel monitoring indicator derived by near-infrared spectroscopy. We hypothesized that tissue oxygenation index could predict a minimum safe flow rate for specific bypass conditions. METHODS: Thirty-six piglets (age, 43 +/- 5 days; weight, 9.0 +/- 1.1 kg) underwent cardiopulmonary bypass with cerebral near-infrared spectroscopy (NIRO-300; Hamamatsu Photonics K.K., Hamamatsu City, Japan). Animals were cooled for 40 minutes to 15 degrees C, 25 degrees C, or 34 degrees C (pH-stat, hematocrit value of 20% or 30%, and pump flow of 100 mL . kg -1 . min -1), followed by low-flow perfusion (10, 25, or 50 mL . kg -1 . min -1) for 2 hours. Neurologic and behavioral evaluations were determined for 4 days. The brain was then fixed for histologic assessment. Tissue oxygenation index was defined as the average signal during low-flow bypass. RESULTS: Animals with an average tissue oxygenation index of less than 55% showed cerebral injury, whereas animals with an index of greater than 55% showed minimal or no evidence of injury. Correlations were found between average tissue oxygenation index and histologic score (Spearman rho = -0.65, P < .001) and neurologic deficit score (Pearson r = -0.50, P = .002) on the first postoperative day. Temperature (P < .001), flow rate (P < .001), and hematocrit value (P = .002) were multivariable predictors of tissue oxygenation index, as determined by means of multivariable analysis of variance. CONCLUSION: Tissue oxygenation index is a useful monitor for defining the minimum safe flow rate during cardiopulmonary bypass. An index value of less than 55% is a strong predictor of neurologic injury.

Animals↗

PubMed2004

Hemodilution elevates cerebral blood flow and oxygen metabolism during cardiopulmonary bypass in piglets.

BACKGROUND: Hemodilution continues to be widely used during cardiopulmonary bypass (CPB) for both adults and children. Previous studies with nonbypass models have suggested that an increase in cerebral blood flow (CBF) compensates for the reduced oxygen-carrying capacity; however, this increased CBF is achieved by an increase in cardiac output. We hypothesized that even with the fixed-flow perfusion of CPB, CBF would be increased during hemodilution. METHODS: Two experiments were conducted and analyzed separately. In each experiment, 10 piglets were randomized to two different groups, one with a total blood prime yielding a high hematocrit (25% or 30%), and the other with a crystalloid prime resulting in a low hematocrit (10% or 15%). Animals were cooled with pH-stat strategy at full flow (100 or 150 mL.kg(-1).min(-1)) to a nasopharyngeal temperature of 15 degrees C, a period of low flow (50 mL.kg(-1).min(-1)) preceding deep hypothermic circulatory arrest (45 or 60 minutes), and a period of rewarming at full flow. Cerebral blood flow was measured at the beginning of CPB, at the end of cooling, at the end of low flow, 5 minutes after the start of rewarming, and at the end of rewarming by injection of radioactive microspheres. RESULTS: Mean arterial pressure was significantly greater with higher hematocrit at each time point (p< 0.05). Cerebral blood flow and the cerebral metabolic rate of oxygen decreased during cooling and further during low flow bypass but were significantly greater with lower hematocrit during mild hypothermia and at the end of rewarming (p< 0.05). CONCLUSIONS: Hemodilution is associated with decreased perfusion pressure, increased CBF and increased the cerebral metabolic rate of oxygen during hypothermic CPB.

Animals↗

PubMed2004

Emergency endovascular stent-grafting for life-threatening acute type B aortic dissections.

BACKGROUND: There is still a considerable controversy regarding optimal treatment for patients with acute type B aortic dissection. Patients with complicated disease are particularly challenging for cardiovascular treatment. Early surgery for acute dissections of the descending aorta with life-threatening complications is known to carry a high mortality. Endovascular stent grafting is developing as an alternative treatment mainly for chronic stages of type B aortic dissection. It is not clear whether endovascular stent grafting is safe and effective in emergency treatment of acute type B aortic dissection. METHODS: In 10 patients (7 men, 3 women; mean age, 59.2 years; range, 46 to 65 years), endovascular stent grafting was performed within 11.0 +/- 5.9 hours (range, 4 to 24 hours) of diagnosis of complications. Indications for acute intervention included contained rupture, hematothorax, life-threatening malperfusion, and refractory pain. Using a retrograde endovascular route after surgical exposure of the femoral artery, self-expanding stent prostheses consisting of polyester-covered Nitinol (Talent, World Medical; mean diameter, 40 +/- 4 mm; length, 10 cm) were placed into the descending aorta distal to the subclavian artery. Before discharge and on follow-up visits, imaging of the aorta was performed using computed tomography. RESULTS: In 9 of 10 patients (90%), the primary entry could be completely occluded with the endovascular stent. Early mortality was 20% (2 of 10): 1 patient died after disruption of the intimal layer distal to the stent, and 1 patient died in hemorrhagic shock after surgical fenestration of the abdominal aorta for persistent malperfusion. Three patients (30%) required consecutive surgical treatment: indications included acute development of retrograde type A aortic dissection, acute stent dislocation by fractured wires and secondary leakage, and late formation of an aneurysm of the descending aorta 6 months after endovascular stent grafting. There were no surgical or late deaths. CONCLUSIONS: Our experience provides some evidence that early mortality of life-threatening acute type B aortic dissection may be reduced by emergency endovascular stent grafting and that this form of treatment is a promising therapeutic option. Refinements, especially in stent design and application, may further improve the prognosis of patients in the life-threatening situation of complicated acute type B aortic dissection.

Acute Disease↗

PubMed2004

Interaction of temperature with hematocrit level and pH determines safe duration of hypothermic circulatory arrest.

OBJECTIVE: Previous studies have demonstrated that both hematocrit level and pH influence the protection afforded by deep hypothermic circulatory arrest. The current study examines how temperature modulates the effect of hematocrit level and pH in determining a safe duration of circulatory arrest. The study also builds on previous work investigating the utility of near-infrared spectroscopy as a real-time monitor of cerebral protection during circulatory arrest. METHODS: Seventy-six piglets (9.3 +/- 1.2 kg) underwent circulatory arrest under varying conditions with continuous monitoring by means of near-infrared spectroscopy (hematocrit level of 20% or 30%; pH-stat or alpha-stat strategy; temperature of 15 degrees C or 25 degrees C; arrest time of 60, 80, or 100 minutes). Neurologic recovery was evaluated daily by a veterinarian, and the brain was fixed in situ on postoperative day 4 to be examined on the basis of histologic score in a blinded fashion. RESULTS: Multivariable analysis of total histologic score revealed that higher temperature, lower hematocrit level, more alkaline pH, and longer hypothermic circulatory arrest duration were predictive of more severe damage to the brain (P <.01). Regression modeling revealed that higher temperature exacerbated the disadvantage of a lower hematocrit level and longer arrest times but not pH strategy. Normalized oxyhemoglobin nadir time, derived from near-infrared spectroscopy, was positively correlated with neurologic recovery on the fourth postoperative day and with total histologic injury score (P <.0001). CONCLUSION: Hematocrit level and pH, as well as temperature, determine the safe duration of hypothermic circulatory arrest. Near-infrared spectroscopy is a useful real-time monitor of safe duration of circulatory arrest.

Animals↗

PubMed2004

Cerebral ischemia caused by obstructed superior vena cava cannula is detected by near-infrared spectroscopy.

OBJECTIVE: Bicaval venous cannulation is being used with increasing frequency in neonates and infants to avoid circulatory arrest. However, superior vena cava (SVC) cannula obstruction may result in cerebral ischemia with no change in blood pressure or mixed venous O2 saturation. The authors hypothesized that near-infrared spectroscopy (NIRS) would allow noninvasive detection of SVC cannula obstruction. METHODS: Fifteen Yorkshire piglets (9.07 +/- 0.20 kg) underwent total cardiopulmonary bypass (CPB) (100 mL/kg/min, pH-stat strategy, hematocrit of 20%) with ascending aortic and bicaval cannulations. Femoral arterial and SVC pressure were monitored as well as mixed venous O2 saturation. NIRS monitoring of tissue oxygenation index (TOI) as well as oxyhemoglobin and deoxyhemoglobin (HHb) was undertaken. Animals were cooled to an esophageal temperature of 25 degrees C over 20 minutes. CPB flow was reduced to 50 mL/kg/min for 20 minutes. Animals then underwent a 60-minute study period of continuous CPB at 50 mL/kg/min with manipulation of the SVC cannula: group 1, open; group 2, partial occlusion; and group 3, complete occlusion. Animals were rewarmed to 37 degrees C at full flow with the SVC cannula open. Cerebral blood flow was assessed at onset of CPB, at end of cooling, at end of low flow, at end of SVC manipulation period, and at end of rewarming using radioactive microspheres. RESULTS: CBF decreased to 27.9 +/- 1.5 mL/min/100 g with complete occlusion (p < 0.01 v group 1: 39.7 +/- 1.9, group 2, 38.3 +/- 2.0 mL/min/100 g) with no change in arterial pressure or mixed venous saturation. There were also significant differences in cerebral oxygen delivery between group 3 and other groups (p < 0.01). SVC pressure increased to 19.5 +/- 4.5 and 32.5 +/- 3.1mmHg with partial and complete occlusion. NIRS indicated significant cerebral ischemia with a decrease in TOI (p < 0.05; group 3 v group 1 and 2) and an increase in HHb (p < 0.05; group 3 v group 1). At the end of the study, significant acidosis was found in group 3 compared with group 1 (p < 0.05). CONCLUSION: SVC cannula obstruction causes cerebral ischemia with no change in blood pressure or venous oxygen saturation. In view of the difficulties and risks of CVP monitoring in babies, it is recommended to use other monitoring modalities such as NIRS to assess adequacy of cerebral perfusion if bicaval cannulation is used in neonates and infants.

Animals↗

PubMed2004

Effects of hemodilution and phenylephrine on cerebral blood flow and metabolism during cardiopulmonary bypass.

OBJECTIVE: Hypotension resulting from hemodilution on cardiopulmonary bypass is often treated by pressor (eg, phenylephrine) infusion. The effect of phenylephrine on cerebral blood flow (CBF) in this setting is not clear. It was hypothesized that phenylephrine might decrease CBF. MEASUREMENTS AND MAIN RESULTS: Six different radioactively labeled microspheres (15 microm) were used to measure CBF at 6 time points (T) in 9 pigs (mean body weight 11.3 +/- 1.2 kg): T1 baseline before bypass (mean arterial pressure [MAP] 76 +/- 5 mmHg), T2 on mildly hypothermic CPB (34 degrees C, pump flow 100 mL/kg/min, hematocrit 30%, MAP 79 +/- 7 mmHg), T3 after moderate hemodilution with crystalloid (hematocrit 20%, resulting MAP 62 +/- 6 mmHg), T4 after phenylephrine administration to increase MAP to baseline values (hematocrit 20%), T5 after severe hemodilution (hematocrit 10%, resulting MAP 41 +/- 4 mmHg), and T6 after phenylephrine administration to normalize MAP (hematocrit 10%). In addition, blood flow to liver, small bowel and skeletal muscle, and pH of jugular venous blood were measured at each time point. After institution of CPB, the CBF (mL/min/100 g tissue) increased significantly to 53 +/- 9 (baseline levels 44 +/- 8, T1 v T2, p = 0.03). Hemodilution resulted in significant increases in CBF on CPB to 65 +/- 9 and 90 +/- 9 at hematocrit 20% and hematocrit 10%, respectively (T2 v T3, p = 0.03; T3 v T5, p = 0.01) and a progressive fall in jugular venous pH. At each level of hemodilution, phenylephrine resulted in an additional increase in CBF (T4, 74 +/- 8; T6, 108 +/- 12; T3 v T4, p = 0.04; T5 v T6, p = 0.01) but did not improve jugular venous pH. Changes in liver blood flow after hemodilution and vasopressor injection showed a similar pattern to CBF. However, the blood flow to small bowel and skeletal muscle increased with hemodilution but decreased significantly with phenylephrine administration. CONCLUSIONS: Phenylephrine redirects blood flow from the bowel and muscle to the brain and liver. Hemodilution increases CBF and pressor administration further increases CBF by elevating perfusion pressure. Maintenance of a higher hematocrit on CPB increases MAP and should decrease the need for vasopressor administration.

Animals↗

PubMed2003

Direct visualization of minimal cerebral capillary flow during retrograde cerebral perfusion: an intravital fluorescence microscopy study in pigs.

BACKGROUND: Retrograde cerebral perfusion (RCP) is used in some centers during aortic arch surgery for brain protection during hypothermic circulatory arrest. It is still unclear however whether RCP provides adequate microcirculatory blood flow at a capillary level. We used intravital microscopy to directly visualize the cerebral capillary blood flow in a piglet model of RCP. METHODS: Twelve pigs (weight 9.7 +/- 0.9 kg) were divided into two groups (n = 6 each): deep hypothermic circulatory arrest (DHCA) and RCP. After the creation of a window over the parietal cerebral cortex, pigs underwent 10 minutes of normothermic bypass and 40 minutes of cooling to 15 degrees C on cardiopulmonary bypass ([CPB] pH-stat, hemocrit 30%, pump flow 100 mL x kg(-1) x min(-1)). This was followed by 45 minutes of DHCA and rewarming on CPB to 37 degrees C. In the RCP group the brain was retrogradely perfused (pump flow 30 mL x kg(-1) x min(-1)) during DHCA through the superior vena cava after inferior vena cava occlusion. Plasma was labeled with fluorescein-isothiocyanate-dextran for assessing microvascular diameter and functional capillary density (FCD), defined as total length of erythrocyte-perfused capillaries per observation area. Cerebral tissue oxygenation was determined by nicotinamide adenine dinucleotide hydrogen (NADH) autofluorescence, which increases during tissue ischemia. RESULTS: During normothermic and hypothermic antegrade cerebral perfusion the FCD did not significantly change from base line (97% +/- 14% and 96% +/- 12%, respectively). During retrograde cerebral perfusion the FCD decreased highly significantly to 2% +/- 2% of base line values (p < 0.001). Thus there was no evidence of significant capillary blood flow during retrograde cerebral perfusion. The microvascular diameter of cerebral arterioles that were slowly perfused significantly decreased to 27% +/- 6% of base line levels during RCP. NADH fluorescence progressively and significantly increased during RCP, indicating poorer tissue oxygenation. At the end of retrograde cerebral perfusion there was macroscopic evidence of significant brain edema. CONCLUSIONS: RCP does not provide adequate cerebral capillary blood flow and does not prevent cerebral ischemia. Prolonged RCP induces brain edema. However, there might be a role for a short period of RCP to remove air and debris from the cerebral circulation after DHCA because retrograde flow could be detected in cerebral arterioles.

Animals↗

PubMed2002

Effects of pH management during deep hypothermic bypass on cerebral microcirculation: alpha-stat versus pH-stat.

OBJECTIVE: There is controversy regarding the optimal pH strategy during deep hypothermic bypass in children. We directly visualized the effects of the pH-stat and alpha-stat strategy on cerebral microcirculation (including leukocyte/endothelial cell interactions) in a piglet model using intravital fluorescence microscopy. METHODS: Two groups of 5 piglets (mean weight 9.6+/-1.3 kg) with a cranial window over parietal cerebral cortex underwent 10-minute normothermic bypass, 40-minute cooling on cardiopulmonary bypass ([CPB] Hct 30%, 100 mL/kg/min), 60-minute circulatory arrest at 15 degrees C, and 40-minute rewarming with alpha-stat (group alpha) or pH-stat (group pH). Plasma was labeled with fluorescein-ITC-dextran for assessment of microvascular diameter. Circulating leukocytes were labeled and observed in postcapillary venules for adhesion before and up to 120 minutes after CPB. Cerebral tissue oxygenation was evaluated by quantification of NADH autofluorescence, which increases during ischemia. RESULTS: At the end of normothermic bypass diameter of cerebrocortical microvessels increased to 116+/-9% (alpha) versus 119+/-10% (pH) of pre-CPB baseline values. During cooling microvascular diameter decreased in group alpha and significantly increased in group pH (89+/-11% (alpha) versus 132+/-13% (pH) at the end of cooling; P<0.001). During the first 10 minutes of rewarming, the cerebral microvascular diameter was significantly larger when the pH stat strategy was used. Tissue oxygenation at the end of cooling was significantly greater in the pH-stat group (P=0.008). On reperfusion, the pH-stat strategy resulted in significantly more rapid return of tissue oxygenation toward baseline although at the end of rewarming the metabolic recovery was complete in both groups. The whole body lactate during early rewarming was significantly less with the pH stat strategy. There was no significant difference between the groups regarding the number of adherent leukocytes throughout the time course of the experiment. CONCLUSIONS: pH-stat management increases tissue oxygenation during deep hypothermic bypass and after circulatory arrest. Leukocyte/endothelial cell interactions during hypothermic bypass are mild with both alpha-stat and pH-stat.

Animals↗

PubMed2002

Dopamine treatment of postischemic contractile dysfunction rapidly induces calcium-dependent pro-apoptotic signaling.

BACKGROUND: Ischemia and adrenergic stimulation of cardiomyocyte cultures have been shown to induce apoptotic cell death. We hypothesized that in a model of contractile dysfunction following ischemia, a commonly used catecholamine such as dopamine augments cardiomyocyte apoptosis via activation of calcium-dependent signaling cascades. METHODS AND RESULTS: Isolated perfused rabbit hearts were subjected to 45 minutes of normothermic ischemia with cardioplegic arrest. Hearts were reperfused for 120 minutes with unmodified perfusate (control), perfusate containing 20 nM dopamine, dopamine+2,3-butanedione monoxime (BDM), a MgATPase-inhibitor, or the calcium-sensitizing inotrope ORG 30029. Ischemia-reperfusion alone caused contractile dysfunction without significant myocardial necrosis (left ventricular pressure-volume curves; 1% triphenyltetrazolium chloride staining; creatine kinase release) or apoptosis (terminal deoxynucleotidyl transferase-mediated nick end labeling [TUNEL] analysis; immunoblotting for poly(ADP-ribose) polymerase [PARP] cleavage; activation of caspases-3, -8, and -9; expression of Bax/Bcl-2). Intracellular calcium [Ca2+]i measured by rhod-2 spectrofluorometry was increased in dopamine-reperfused hearts. Although postischemic dopamine treatment improved contractility, the number of apoptotic cardiomyocytes was significantly higher than in untreated postischemic hearts (32.5+/-9 versus 5.5+/-1.6/1000 nuclei, P<0.01). Further evidence of dopamine-stimulated apoptosis included PARP cleavage, activation of mitochondrial-derived caspase-9, and the terminal effector caspase-3. Dopamine also increased cellular content of pro-apoptotic Bax while decreasing anti-apoptotic Bcl-2. Simultaneous treatment with BDM suppressed contractility without affecting [Ca2+]i and did not reduce dopamine-stimulated apoptotic markers. When contractility was increased without elevating [Ca2+]i using ORG 30029, no activation of pro-apoptotic signaling cascades was found. Dopamine infusion in nonischemic hearts did not result in cardiomyocyte apoptosis. CONCLUSIONS: Postischemic dopamine treatment of contractile dysfunction activates pro-apoptotic signal cascades, most likely via a calcium-dependent process and mitochondrial damage.

Animals↗

PubMed2002

Combination of alpha-stat strategy and hemodilution exacerbates neurologic injury in a survival piglet model with deep hypothermic circulatory arrest.

BACKGROUND: The optimal pH strategy and hematocrit during cardiopulmonary bypass with deep hypothermic circulatory arrest (DHCA) remain controversial. We studied the interaction of pH strategy and hematocrit and their combined impact on cerebral oxygenation and neurological outcome in a survival piglet model including monitoring by near-infrared spectroscopy (NIRS). METHODS: Thirty-six piglets (9.2+/-1.1 kg) underwent DHCA under varying conditions with continuous monitoring by NIRS (pH-stat or alpha-stat strategy, hematocrit 20% or 30%, DHCA time 60, 80, or 100 minutes). Neurological recovery was evaluated daily. The brain was fixed in situ on postoperative day 4 and a histological score (HS) for neurological injury was assessed. RESULTS: Oxygenated hemoglobin (HbO2) and total hemoglobin signals detected by NIRS were significantly lower with alpha-stat strategy during cooling (p < 0.001), suggesting insufficient cerebral blood supply and oxygenation. HbO2 declined to a plateau (nadir) during DHCA. Time to nadir was significantly shorter in lower hematocrit groups (p < 0.01). Significantly delayed neurologic recovery was seen with alpha-stat strategy compared with pH-stat (p < 0.05). The alpha-stat group had a worse histological score compared with those assigned to pH-stat (p < 0.001). Neurologic impairment was estimated to be over 10 times more likely for animals randomized to alpha-stat compared with pH-stat strategy (odds ratio = 10.7, 95% confidence interval = 3.8 to 25.2). CONCLUSIONS: Combination of alpha-stat strategy and lower hematocrit exacerbates neurological injury after DHCA. The mechanism of injury is inadequate cerebral oxygenation during cooling and a longer plateau period of minimal O2 extraction during DHCA.

Animals↗

PubMed2002

Improving results of the modified Fontan operation in patients with heterotaxy syndrome.

BACKGROUND: Historically the Fontan operation in patients with single ventricle heterotaxy syndrome and atrial isomerism has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 135 patients with heterotaxy syndrome who underwent a Fontan operation between 1981 and 2000 was performed. RESULTS: There were 93 patients with right isomerism and 42 with left isomerism. Anomalies of venous return included 25 patients with extracardiac pulmonary venous connection (19%) and 37 patients with an interrupted inferior vena cava (27%). Thirty-six patients (27%) had at least moderate atrioventricular valve regurgitation. The type of Fontan procedure included 17 patients with an atriopulmonary Fontan connection, 67 with a lateral tunnel modification, 19 with an intraatrial tube graft, 25 with an extracardiac tubegraft, and 7 with an intra-extra atrial tube graft. A fenestration was placed in 93 patients (78%). Early mortality was 19% before 1991, 3% since 1991, and no patient has died early since 1993. Ten-year survivals were 70% for Fontan operations before 1990 and 93% for Fontan operations after 1990. Thirty-two patients (23%) had prolonged pleural effusions. Risk factors for death included anomalous pulmonary venous connection (p = 0.02) and higher preoperative pulmonary vascular resistance (p = 0.002). Sixty-two patients (47%) had some form of early postoperative arrhythmia. At 10 years, freedom from late bradyarrhythmia and late tachyarrhythmia were 78% and 70%, respectively. Preoperative arrhythmias, older age at operation, and anatomic features were each independent predictors of late arrhythmia. CONCLUSIONS: The Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative arrhythmias and prolonged pleural effusions remains significant. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a baffle fenestration contribute to improved outcome.

Abnormalities, Multiple↗