Neonatal repair of atypical double outlet right ventricle.

BACKGROUND: A complex and rare form of double outlet right ventricle needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: To point out retrospectively what could have been done differently in our unique patient. METHODS: Primary repair was arranged in a neonate with double outlet right ventricle (of a non-committed ventricular septal defect type and lack of the outlet septum between the semilunar valves) with right aortic arch and dextro-malposition of great arteries. RESULTS: We managed to achieve intraventricular rerouting via a right ventricular incision concomitantly with the arterial switch maneuver. The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered that the radical approach we chose appeared to be sensible in this particular patient, although some other options could have been available.

A retrospective study of factors associated with persistent delirium.

BACKGROUND: It has been reported that delirium causes various problems. Many researchers have reported the risk factors associated with the onset of delirium; however, there are few reports focused on persistent delirium. This study aimed to identify the risk factors associated with persistent delirium. METHODS: A total of 573 patients hospitalised in Nara Prefecture General Medical Centre from October 2014 through September 2017 who were referred to the psychiatry consultation service were included in this study. Persistent delirium was defined as delirium lasting for 14 days or more. A retrospective study was carried out based on the patients' records. The relationship between various background factors and persistent delirium was statistically analysed. RESULTS: Of the 573 hospitalised patients, 295 were diagnosed as having delirium. Forty-six patients with persistent delirium and 181 patients with nonpersistent delirium were included in this study. Multivariable logistic regression analyses revealed that male gender, opioid analgesics use, non-opioid analgesics use, and low serum sodium were significantly and independently associated with persistent delirium. Ramelteon or trazodone was used significantly more in persistent delirium, although each use was not significant. CONCLUSION: This is the first study to reveal that male gender and use of analgesics were associated with persistent delirium in general hospital. However, as this is a case-control study and may contain bias, future cohort studies and intervention studies are needed. It is also necessary to investigate the relevance of the 'degree of pain' behind the use of analgesics.

CRT in a congenital heart disease patient with interventricular dyssynchrony due to an RV conduction delay.

We encountered a unique pattern of cardiac dyssynchrony in a patient with complex congenital heart disease (heterotaxy syndrome) with a biventricular physiology and systemic left ventricle (LV). On speckle tracking echocardiography, dyssynchrony was not detected within the LV, but was noted in an interventricular fashion between the LV and right ventricle (RV). An electrophysiologic study revealed a conduction delay in the subpulmonary RV. Cardiac resynchronization therapy provided reverse cardiac remodeling and an excellent result in our patient by placing the pacing leads around the dyssynchronous lesion.

Is two-staged repair for truncus arteriosus type A3 unpractical?

BACKGROUND: A complex and rare form of persistent truncus arteriosus needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: We herein describe our surgical strategy of a small infant having this malformation concomitantly with right aortic arch, unusual pulmonary artery branching and a left superior vena cava. METHODS: The patient underwent initially bilateral pulmonary arterial banding followed by the Rastelli type definitive repair. The pulmonary arteries were unified in front of the left superior vena cava, and the right ventricular outflow tract was readily reconstructed. RESULTS: The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered the two-stage approach was sensible in this particular patient.

Preoperative simulation for complex transposition of great arteries using a three-dimensional model.

We utilised a cast preoperatively prepared on the basis of the patient's computed tomography data to clarify unusual orientation of the great arteries (transposition of great arteries, coarctation of the aorta, and abnormally branching central pulmonary arteries) in a 2.2-kg neonate. The three-dimensional model was very useful for designing the surgical procedure - being easy to handle for real and precise simulation of the reparative manoeuvres.

Immediate and Midterm Cardiac Remodeling After Surgical Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study.

BACKGROUND: Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling. METHODS: Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y). RESULTS: There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m2; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m2; P<0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m2 and RVESVi ≤82 mL/m2. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P<0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm2/m2; P<0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (P=0.03) over a median of 9.5 years of follow-up. CONCLUSIONS: Significant right heart structural reverse remodeling takes place immediately after PVR, followed by a continuing process of further biological remodeling manifested by further reduction in RVESVi. PVR before RVESVi reaches 82 mL/m2 confers optimal chances of normalization of RV function.

Persistent fifth aortic arch: the "great pretender" in clinical practice.

Persistence of the embryonic "fifth aortic arch" in postnatal life is a rare, enigmatic - and at times controversial - condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the "great pretender" by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of "persistent fifth aortic arch" while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

Right Ventricular Pressure Overload and Pathophysiology of Growing Porcine Biomodel.

The primary objective was to create a clinically relevant model of right ventricular hypertension and to study right ventricular myocardial pathophysiology in growing organism. The secondary objective was to analyse the effect of oral enoximone (phosphodiesterase inhibitor) therapy on right ventricular haemodynamic parameters and myocardial changes in biomodel of right ventricular hypertension. The study included a total of 12 piglets of 42 days of age. Under general anaesthesia, pulmonary artery banding (PAB) was performed surgically to constrict the main pulmonary artery to about 70-80 % of its original dimension. The study presented two groups of animals labelled C (control animals with PAB; n = 8) and E (animals with PAB and oral administration of enoximone; n = 4). Direct pressure and echocardiographic measurements were taken during operation (time-1), and again at 40 days after surgery (time-2). The animals were killed, and tissue samples from the heart chambers were collected for quantitative morphological assessment. Statistical analysis was performed on all acquired data. At time-2, the median weight of animals doubled and the median systolic pressure gradient across the PAB increased (46.59 ± 15.87 mmHg vs. 20.29 ± 5.76 mmHg; p < 0.001). Changes in haemodynamic parameters were compatible with right ventricular diastolic dysfunction in all the animals. Apoptosis, tissue proliferation and fibrosis were identified in all the myocardial tissue samples. Right ventricular pressure overload leads to increased apoptosis of cardiac myocytes, proliferation and myocardial fibrosis. Our study did not show evidence of haemodynamic benefit or myocardial protective effect of oral enoximone treatment.

The Importance of Walking for Control of Blood Pressure: Proof Using a Telemedicine System.

BACKGROUND: Regular physical activity (PA), including daily walking, reduces the risk of many chronic diseases, especially hypertension. Pedometer is a potential motivational aid for increasing PA. In the present study, we used a telemedicine system and analyzed the relationship between daily walking, calculated by pedometers, and blood pressure (BP). METHODS: BP was measured at home twice a day (morning and evening) using an oscillometric automatic device. Body weight (BW) and percent body fat (%BF) were measured after BP measurement. Daily walking steps (DWS) were calculated by a pedometer. These daily parameters were transmitted through the Internet to a central server computer and sent to the Medical Health Center. RESULTS: Sixty-nine (N = 69) hypertensive patients were included in this study. The mean follow-up period was 378 days. Electronic data from a pedometer (DWS) were associated with reduced BW, body mass index, and %BF. Hypertensive patients were divided into two groups based on the DWS. In the high DWS group, morning systolic BP and diastolic BP and evening systolic BP were reduced after induction of the telemedicine system. CONCLUSION: A telemedicine system confirmed the usefulness of walking to control BP in hypertensive patients.

Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of Fallot and potential prognostic value of preoperative cardiopulmonary exercise testing.

BACKGROUND: Indications for surgical pulmonary valve replacement (PVR) after repair of tetralogy of Fallot have recently been broadened to include asymptomatic patients. METHODS AND RESULTS: The outcomes of PVR in adults after repair of tetralogy of Fallot at a single tertiary center were retrospectively studied. Preoperative cardiopulmonary exercise testing was included. Mortality was the primary outcome measure. In total, 221 PVRs were performed in 220 patients (130 male patients; median age, 32 years; range, 16-64 years). Homografts were used in 117 patients, xenografts in 103 patients, and a mechanical valve in 1 patient. Early (30-day) mortality was 2%. Overall survival was 97% at 1 year, 96% at 3 years, and 92% at 10 years. Survival after PVR in the later era (2005-2010; n=156) was significantly better compared with survival in the earlier era (1993-2004; n=65; 99% versus 94% at 1 year and 98% versus 92% at 3 years, respectively; P=0.019). Earlier era patients were more symptomatic preoperatively (P=0.036) with a lower preoperative peak oxygen consumption (peak Vo2; P<0.001). Freedom from redo surgical or transcatheter PVR was 98% at 5 years and 96% at 10 years for the whole cohort. Peak Vo2, E/CO2 slope (ratio of minute ventilation to carbon dioxide production), and heart rate reserve during cardiopulmonary exercise testing predicted risk of early mortality when analyzed with logistic regression analysis; peak Vo2 emerged as the strongest predictor on multivariable analysis (odds ratio, 0.65 per 1 mL·kg⁻¹·min⁻¹; P=0.041). CONCLUSIONS: PVR after repair of tetralogy of Fallot has a low and improving mortality, with a low need for reintervention. Preoperative cardiopulmonary exercise testing predicts surgical outcome and should therefore be included in the routine assessment of these patients.

Vascular histopathologic reaction to pulmonary artery banding in an in vivo growing porcine model.

Pulmonary artery banding (PAB) is used as a surgical palliation to reduce excessive pulmonary blood flow caused by congenital heart defects. Due to the lack of microscopic studies dealing with the tissue remodeling caused by contemporary PAB materials, this study aimed to assess histologic changes associated with PAB surgery by analyzing local tissue reaction to the presence of Gore-Tex strips fixed around the pulmonary artery. Gore-Tex strips were used for PAB in a growing porcine model. After 5 weeks, histologic samples with PAB (n = 5) were compared with healthy pulmonary arterial segments distal to the PAB or from a sham-treated animal (n = 1). Stereology was used to quantify the density of the vasa vasorum and the area fraction of elastin, smooth muscle actin, macrophages, and nervi vasorum within the pulmonary arterial wall. The null hypothesis stated that samples did not differ histopathologically from adjacent vascular segments or sham-treated samples. The PAB samples had a greater area fraction of macrophages, a lower amount of nervi vasorum, and a tendency toward decreased smooth muscle content compared with samples that had no PAB strips. There was no destruction of elastic membranes, no medionecrosis, no pronounced inflammatory infiltration or foreign body reaction, and no vasa vasorum deficiency after the PAB. All the histopathologic changes were limited to the banded vascular segment and did not affect distal parts of the pulmonary artery. The study results show the tissue reaction of palliative PAB and suggest that Gore-Tex strips used contemporarily for PAB do not cause severe local histologic damage to the banded segment of the pulmonary arterial wall after 5 weeks in a porcine PAB model.

Observation of Atrial Fibrillation Dependent on an Intra-atrial Re-entrant Tachycardia Within the Right Atrium in a Repaired Tetralogy of Fallot.

Atrial fibrillation (AF) can occur predominantly associated with right atrial (RA) lesions in congenital heart disease, particularly when the RA cavity is dilated. RA electrical potentials occasionally appear organized during AF. We clearly mapped such areas circumscribed by an intra-atrial re-entrant circuit during an isoproterenol infusion, in a patient with a repaired tetralogy of Fallot, using an ultrahigh-density mapping system and its beat acceptance criteria function. Ablation of areas inside the re-entrant circuit successfully eliminated the AF. Our experience indicated that a macro-re-entrant tachycardia was a driver as well as a trigger of AF of this right-sided origin.

Chez les patients atteints d'une cardiopathie congénitale, la fibrillation auriculaire (FA) peut souvent survenir en association avec des lésions auriculaires droites (AD), en particulier lorsque la cavité AD est dilatée. Lors d'une FA, il peut arriver que les potentiels électriques AD semblent normaux. Chez un patient ayant une tétralogie de Fallot réparée, nous avons clairement cartographié des zones délimitées par un circuit de réentrée intra-auriculaire lors d'une perfusion d'isoprotérénol, et ce, à l'aide d'un système de cartographie à très haute densité et de ses critères d'acceptation liés aux battements cardiaques. L'ablation des régions se trouvant dans le circuit de réentrée a permis d'éliminer la FA avec succès. Notre expérience a démontré qu'une tachycardie macroréentrante avait été un facteur déterminant et même un déclencheur de la FA, laquelle est apparue à droite.

Perspective of preexcitation induced cardiomyopathy; early septal contraction, and subsequent rebound stretch.

BACKGROUND: Preexcitation-induced cardiomyopathy (PIC) is defined as a disease presenting ventricular dyssynchrony because of preexcitation through an accessory pathway (AP), being a cousin of pacing-induced cardiomyopathy. The present review aims at providing perspective of this uncharted subgroup. METHODS: In order to determine mechanisms and clinical characteristics of PIC, 63 patients in 29 literature reports were reviewed. RESULTS: A median age at onset was 4 (0.1-59) years; 55 patients (87%) under 18 years old including 16 infants. Twenty patients (32%) experienced supraventricular tachycardia prior and subsequent to the PIC onset. Heart failure and left ventricular (LV) dysfunction did not correlate with other clinical features. All the 65 APs identified (duplicated in 2 patients) were located on the right side of the atrioventricular junction; at the septal area (in 55%) or the anterolateral aspect (in 22%). AP conduction was successfully eliminated by medical or interventional treatments where attempted. LV function returned to normal within 6 months in 67% of patients, while recovery took longer than 3 years in 8%. Frequently seen at the basal segments of the interventricular septum were early contraction within the QRS complex, dyskinesis at mid-systole, and aneurysm/bulging or local thinning. CONCLUSIONS: Several characteristic factors became clear as described above. Rebound stretch following early shortening of the interventricular septum is seemingly the major mechanism of PIC, and thus a right septal or right anterior/anterolateral AP needs attention as a higher risk for PIC.

Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study.

BACKGROUND: late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. METHODS AND RESULTS: we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. CONCLUSIONS: preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

Use of hydrocolloid dressing in infants requiring open chest management after cardiac surgery.

PURPOSE: Sternal splintage is known as an effective maneuver to stabilize hemodynamics during the immediate postoperative period, particularly in very sick infants. On the other hand, its wound management is not always straightforward. We employed dressing using a product made of a hydrocolloid material in such circumstances. This report describes our experience in utilizing the dressing in term of its potential advantages. MATERIALS AND METHODS: Six infants needed open chest management following complicated procedures for congenital heart disease. A polytetrafluoroethylene patch was fixed to augment the skin defect at the time of sternal splintage, and a hydrocolloid dressing was applied to entirely cover the wound including the suture line. RESULT: All patients survived their difficult circumstances. None of them suffered wound complications such as infection or healing problem during sternal splintage or subsequent to eventual chest closure. The dressing product was easy to handle with no adverse events associated with its material. CONCLUSIONS: It is reconfirmed that a dressing made of hydrocolloid material was of practical use for sealing the wound in infants requiring open chest management after cardiac surgery.

Cor Triatriatum Can Coexist With Common Pulmonary Venous Atresia.

Surgical treatment was very urgently carried out in an extremely cyanotic newborn with common pulmonary venous atresia. The diagnosis was confirmed during the procedure, and the common venous chamber was anastomosed to the left atrium through a transverse incision onto the right and the left atria. Unfortunately, pulmonary hypertension persisted during the postoperative course and even got worse gradually. Computed tomography eventually illustrated residual cor triatriatum. The initial atriotomy had been entirely above the diaphragm within the left atrium. Consequently, the abnormal structure had been overlooked. The patient is doing well 1 month after the surgical revision. Pulmonary hypertension disappeared.

Etiology of atrial fibrillation in patients with complex congenital heart disease - for a better treatment strategy.

BACKGROUND: The demographics of patients with congenital heart disease (CHD) and atrial fibrillation (AF) differ significantly from the general population. The etiology and treatment strategy for AF in CHD patients have been investigated but are to date inconclusive. METHODS: To determine the etiology of AF in CHD and to seek a better treatment strategy, we retrospectively evaluated the atrial overload in 42 complex CHD cases with normal atrial arrangements and AF (age 25; range, 9-66 years) and the impact of a reduction in the atrial overload on the atrial rhythm. RESULTS: Cardiac defect diagnoses varied, with 17% of the patients having a persistent left superior vena cava (PLSVC). In regard to the volume overload, the frequencies of an overload in the right atrium (RA), left atrium (LA), or both, were 50 %, 23%, and 10%, respectively (p = 0.015). Other sustained supraventricular tachycardias were observed in 29 patients (69%) before and after the onset of AF. Among these 29 patients, 26 had intra-atrial reentrant tachycardia. Fifteen patients (36%), 10 of whom had chronic AF, died during the follow-up including 3 with arrhythmias and 10 because of heart failure. Fourteen (33%) patients had no AF at the last follow-up due to medical interventions, 8 of which underwent solely an RA-sided catheter ablation and/or surgical RA overload reduction. CONCLUSIONS: AF in complex CHD with a normal atrial arrangement correlates with a higher RA-sided overload than an LA-sided and exhibits a high incidence of PLSVCs, high comorbidity of intra-atrial reentrant tachycardias, and high mortality rate. In a substantial number of patients, RA-sided interventions were effective in controlling AF. To effectively manage AF in complex CHD it is essential to understand each individual's hemodynamics and consider hemodynamic interventions.

Major adverse events and atrial tachycardia in Ebstein's anomaly predicted by cardiovascular magnetic resonance.

OBJECTIVES: Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. METHODS: Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT). RESULTS: CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007). CONCLUSION: CMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Preoperative small pulmonary artery did not affect the midterm results of Fontan operation.

OBJECTIVE: It was well discussed, in the primary Fontan era, that small pulmonary arterial size could affect the results immediately after the Fontan procedure. The objective of the present study is to determine whether this feature remains as a risk factor in the era of the staged Fontan completion and poses functional impediments in the longer terms. METHODS: Between June 1991 and November 2004, the staged Fontan completion was carried out subsequent to the bidirectional Glenn procedure in consecutive 57 patients with a preoperative pulmonary artery index less than 250 mm2/m2 (Group-S; minimum index 104 mm2/m2). Clinical data were reviewed retrospectively. As background and reference information, similar data were collected in another consecutive 64 patients with larger pulmonary arteries who underwent the staged Fontan completion during the same period (Group-L; maximum index 697 mm2/m2). RESULTS: No patients died after the Fontan procedure in Group-S, while six early deaths in Group-L. No takedown of the Fontan circulation was carried out in either group. The latest catheterizations, at 2.8+/-2.7 years postoperatively, showed a pulmonary artery index significantly lower than the preoperative index (Group-S: 198+/-37-176+/-49 mm2/m2; P=0.0082, Group-L: 360+/-94-266+/-89 mm2/m2; P<0.0001). Hemodynamics in Group-S during the intermediate term were identical with those in Group-L in mean pulmonary arterial pressure (10+/-2 in Group-S and 10+/-3 mmHg in Group-L), mean atrial pressure for the systemic chambers (5+/-2 and 6+/-3 mmHg, respectively), mean transpulmonary gradient (5+/-2 and 4+/-2 mmHg, respectively), cardiac index (3.0+/-0.7 and 3.0+/-0.6l/min/m2, respectively), and arterial oxygen saturation (93+/-3% and 94+/-2%, respectively). Similarly, brain natriuretic peptides concentration in the serum (19.4+/-15.6 in Group-S and 28.3+/-37.2 pg/ml in Group-L) and peak oxygen consumption on exercise testing (24.8+/-4.5 and 24.0+/-6.3 ml/kg/min, respectively) were not inferior in Group-S to those in Group-L. CONCLUSIONS: The outcome after the Fontan completion, including functional ones in the intermediate term, was acceptable in patients having a preoperative PA index smaller than 250 mm2/m2. Pulmonary artery index decreased still further postoperatively, but did not obviously militate against functional efficacy of the Fontan circulation.