Included Tricuspid Atresia Research Articles

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients' original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.

Juxtaposition of the Atrial Appendages: A Large Echocardiographic Series

Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions. This is the largest echocardiographic study of juxtaposition of the atrial appendages. This is a retrospective study using the Mt. Sinai echocardiogram database (EchoLAN), which contains echocardiogram reports performed or reviewed at Mt. Sinai Hospital (New York, NY) between 1992 and 2019. Each report was reviewed for associated intracardiac and extracardiac anomalies and the prevalence of juxtaposed atrial appendage among specific diagnosis, including tricuspid atresia, transposition of the great arteries, and double outlet right ventricle, was calculated. Descriptive and analytical statistics were performed as applicable, including Fisher's exact test, with p value < 0.05 considered statistically significant. Forty-nine patients had juxtaposed atrial appendages (1.2/1000 patients): Thirty-eight had LJRAA (0.9 per 1000 patients) and eleven had RJLAA (0.3 per 1000 patients). LJRAA was seen in 22% of tricuspid atresia, 6% of d-TGA, 1% of l-TGA, and 5% of DORV. RJLAA was seen in 2% of DORV, and none of the other lesions. Of associated lesions, dextrocardia, mesocardia, transposition, tricuspid atresia, double inlet left ventricle, and pulmonary valve stenosis were more likely to be present in LJRAA, while bicuspid aortic valve and left ventricular hypoplasia were more likely to be present in RJLAA. This study reaffirms our understanding of juxtaposition of the atrial appendages, its prevalence, and clinical importance.

Fontan procedure with extracardiac conduit in pediatrics and children: Short-term results

IntroductionThe concept of Fontan circulation was first clinically introduced in 1971. Technical modifications have been advocated with the concept of total cavopulmonary connection, introduced in the late 80s. Recently, an extracardiac conduit to divert inferior vena cava flow to the pulmonary arteries has been advocated, and it affords excellent palliation for patients with various forms of the anatomic or functional single ventricle.Patients and methodsFrom September 2019 to August 2021, 12 patients (five males and seven females) with a mean age of 10.7 (6–15) years underwent a Fontan procedure with extracardiac conduit at the National Heart Institute. The mean weight was 34.75 (18–60) kg. The underlying diagnoses included tricuspid atresia (n = 2), double-inlet left ventricle (n = 3), transposition of great arteries (n = 1), and double-outlet right ventricle (n = 6). All patients were in sinus rhythm.ResultsThere was one operative mortality. The type of conduit used was Gortex in 11 patients (91.7%) and Dacron in one patient (8.3%). The mean conduit size was 20 mm (16–26 mm). The mean cardiopulmonary bypass time was 127 (90–200) min. Aortic cross-clamping time was 25 min in two patients who needed total circulatory arrest. The mean duration of chest tube drainage was 31.8 days (15–130 days). All patients had sinus rhythm before the operation remained sinus. The mean ejection fraction was 65% (55–71%). The arterial oxygen saturation increased from a preoperative mean level of 64.2% (55–70%) to 90% (85–93%). There were nine patients (75%) who had fenestrations. The mean postoperative hospital stay was 36.7 (15–150) days, ICU stay was 6.5 (3–8) days, and mechanical ventilation time was 13.7 (4–27) h. There were three patients (25%) who needed reoperation, one who needed reintervention with chylous pleural effusion (8.3%), protein-losing enteropathy (8.3%), and infection. All patients had New York Heart Association (NYHA) functional class ≤II.ConclusionThe extracardiac conduit is an easy way to perform the Fontan procedure, as it provides an excellent early result, maintenance of sinus rhythm, and preservation of ventricular function.

Strain and Rotational Mechanics in Children With Single Left Ventricles After Fontan

Left ventricular (LV) mechanics in patients with different single morphologic LV subtypes, including tricuspid atresia, double-inlet left ventricle, and pulmonary atresia with intact ventricular septum, remain poorly studied. Given that histologic studies indicate differences in LV myocardial fiber orientation, we hypothesized that this may result in altered LV mechanics. The aim of this study was to evaluate the influence of LV morphology on LV mechanics. Fifty-two children with single left ventricles after Fontan operation and age-matched control subjects were prospectively enrolled. Using two-dimensional speckle-tracking echocardiography, longitudinal strain was measured in the four-, three-, and two-chamber long-axis planes, and circumferential strain was measured at the basal, mid, and apical short-axis planes. Apical and basal rotation were measured, and twist and torsion were calculated. We compared strain and rotational mechanics in cases versus control subjects and among LV subtypes. Compared with control subjects, subjects with single left ventricles had similar LV end-diastolic dimensions but significantly decreased ejection fractions. The single left ventricle cohort had normal global longitudinal strain (P=.20) but lower basal mean circumferential strain (P<.0001). Single left ventricle subjects had higher apical rotation (P=.0001) but decreased basal rotation (P=.0007); there was no difference in twist but increased torsion (P=.001). LV subtypes had different four-chamber (P=.01), two-chamber (P=.006), and global longitudinal strain (P=.01), lowest in the pulmonary atresia with intact ventricular septum subtype. Longitudinal LV strain was preserved in children with single left ventricles after Fontan. A pattern of reduced basal circumferential strain and rotation with an increase in apical rotation and torsion in the single left ventricle cohort may be related to differences in myofiber orientation, increased fibrosis, and the impact of altered loading conditions throughout palliation. Decreased longitudinal strain in the pulmonary atresia with intact ventricular septum group may also reflect detrimental interventricular interactions.

EVALUATING PULMONARY EMBOLISM IN PATIENTS WITH FONTAN PHYSIOLOGY

Fontan surgery results in complex cardiopulmonary anatomy which complicates interpretation of computed tomography angiography (CTA) when evaluating for pulmonary embolism (PE). A 42-year-old man presented with acute dyspnea and pleuritic chest pain. Medical history included tricuspid atresia status

Quality of Life for Historic Cavopulmonary Shunt Survivors.

Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort. Of the original 91 cavopulmonary shunt survivors, 14 (ages: 26-59; average length of postsurgical follow-up: 38.4 years) completed the Rand 36-Item Short-Form Health Survey, volume 2 (SF-36v2) quality-of-life survey at clinical office visits, over the phone or via regular mail. Diagnoses included tricuspid atresia (n = 9), double inlet left ventricle (n = 1), d-transposition of great arteries (n = 1), Ebstein's anomaly (n = 1), tetralogy of Fallot (n = 1), and pulmonary atresia with hypoplastic right ventricle (n = 1). Norm-based comparison of cavopulmonary shunt survivors to the general population was performed using a one-sample t test. The 36-question health survey, SF-36v2, provided physical and mental health summary measures standardized to achieve a mean of 50. The 14 survivors' physical composite score was 47.18 (standard deviation [SD]: 8.24; P = .22) and mental composite score was 52.71 (SD: 5.64; P = .095). The cavopulmonary shunt is now a widely used surgical palliation for single-ventricle patients and can be associated with physical and mental health outcomes similar to the general US population. Cavopulmonary shunt survivors' SF-36v2 survey results do not demonstrate a statistically significant difference from the general US population in physical or mental health measures. The results of this study contribute to the discussion of quality of life for patients with congenital heart disease by following up with the oldest known survivors.

Pseudoaneurysm Development After Ablation for Atrial Tachycardia in a PatientWith Single-Ventricle Anatomy.

A 39-year-old woman with complex congenital heart disease presented with refractory atrial arrhythmias unresponsive to multiple antiarrhythmic agents. Her cardiac anatomy included tricuspid atresia with a single left ventricle, severe pulmonary valve stenosis and transposed great arteries, and

Intraatrial Conduit Fontan Procedure: Indications, Operative Techniques, and Clinical Outcomes

The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg. The cardiac malformations included tricuspid atresia in 10, double-inlet left ventricle in 8, double-inlet right ventricle in 15, double-outlet right ventricle in 18, congenitally corrected transposition of the great arteries in 11, complete atrioventricular septal defect in 8; criss-cross in 1, pulmonary atresia with intact ventricular septum in 3, Ebstein anomaly in 3, and others in 3. Heterotaxy syndrome was found in 21 patients. An intraatrial polytetrafluoroethylene conduit was implanted to construct the Fontan pathway. In 75 patients, a 2.7-mm to 4.5-mm fenestration was made to reduce the intraconduit pressure. Eighty-three patients had previously undergone a Glenn operation. Median follow-up was 29 months (range, 2 to 60 months). Doppler echocardiography, electrocardiography, and Holter monitoring were used to evaluate hemodynamic performance and arrhythmias. There were 2 hospital deaths, 1 Fontan takedown, and 3 midterm deaths. Conduit thrombosis developed in 1 patient. Atrial flutter developed in 2 patients, who underwent electrical cardioversion. Junctional bradycardia developed in 2 patients, but they did not require permanent pacemakers. Overall survival was 97.0% at 1 year and 94.1% at 5 years. The IAC modification provides excellent operative and midterm outcomes in most patients with a functional single ventricle. However, a longer follow-up time is required to demonstrate its real advantages.

Fontan Conversion: Identifying the High-Risk Patient

Patients with atriopulmonary Fontan tend to undergo conversion to total cavopulmonary connections secondary to arrhythmias or poor flow dynamics. However, the ideal candidate is unknown. Between December 1994 and May 2011, 70 patients (40 males [57%]) underwent Fontan conversion. Median age was 23 years (range, 4 to 46 years). Excluded were 1.5 ventricle conversions. The most common diagnoses included tricuspid atresia in 34 patients (49%) and double-inlet left ventricle in 16 (23%). Atrial tachyarrhythmia was present in 62 patients (89%), 41 (59%) had atrioventricular valve (AVV) regurgitation, and 32 (46%) were in New York Heart Association class III or IV. Atriopulmonary Fontan was the original connection in 58 patients (83%), whereas the Björk modification was performed in 8 (11%). Fontan was performed with an intraatrial conduit in 41 patients, an extracardiac conduit in 18, and a lateral tunnel in 11. Forty-nine patients (70%) underwent concomitant arrhythmia operations. Early death occurred in 10 patients (14%). Multivariate analysis revealed age older than 27 years (p = 0.009), AVV regurgitation (p = 0.016), lack of arrhythmia operation (p = 0.04), and male sex (p = 0.02) were predictors of perioperative death. Mean follow-up was 5 years (maximum, 17 years). Overall survival at 1, 5, and 10 years was 81%, 70%, and 67%, respectively, and 84% of patients were in New York Heart Association class I or II. Proper selection of Fontan conversion candidates is critical. Concomitant arrhythmia operations may be associated with improved survival. Older age and AVV regurgitation increase the risk of poor outcome, and cardiac transplantation may be a better option.

The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure

The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. It is performed to treat several complex congenital heart abnormalities including tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle. The original Fontan procedure included direct anastomosis of the right atrium to the main pulmonary artery; however, multiple modifications have been employed. Creation of Fontan circulation is palliative in nature, with good results in patients with ideal hemodynamics and substantial morbidity and mortality in those with poor hemodynamics. Complications of Fontan circulation include exercise intolerance, ventricular failure, right atrium dilatation and arrhythmia, systemic and hepatic venous hypertension, portal hypertension, coagulopathy, pulmonary arteriovenous malformation, venovenous shunts, and lymphatic dysfunction (eg, ascites, edema, effusion, protein-losing enteropathy, and plastic bronchitis). Magnetic resonance imaging is best for postoperative evaluation of patients who underwent the Fontan procedure, and cardiac transplantation remains the only definitive treatment for those with failing Fontan circulation.

In situ pericardial extracardiac lateral tunnel Fontan operation: Fifteen-year experience

The study purpose is to evaluate the long-term outcome of the in situ pericardial extracardiac lateral tunnel Fontan operation. From June 1994 to August 2009, 160 patients with single ventricle (boys, n=96, 60%, median age=39 months, mean weight 15.5 kg) underwent the pedicled pericardial extracardiac lateral tunnel operation. Patients' charts were reviewed for perioperative and long-term follow-up data, outcome, and mortality. The potential growth of these tunnels was evaluated. The main diagnoses included tricuspid atresia (n=44, 27%); double-outlet right ventricle (n=29, 18%), and hypoplastic left heart syndrome (n=26, 16%). The mean follow-up was 6.5± 3.7 years (range: 0.1-15 years). There were 2 (1.3%) operative and 6 (3.7%) late deaths. Actuarial survival at 14 years was 93%. Early complications included prolonged effusions (n=35, 22%), chylothorax (n=5, 3.1%), readmissions (n=35, 22%), cerebrovascular accidents (n=8, 5%), contralateral phrenic nerve palsy (n=1, 0.8%), and transient arrhythmias (n=5, 3.1%). No pacemaker was needed. Late complications included tunnel stenosis (n=3, 1.8%) managed with balloon dilatation and stenting in 2 patients and surgical revision in 1; tunnel thrombosis (n=2, 1.2%) causing death in both patients; and protein losing-enteropathy (n=4, 2.5%). Follow-up echocardiography of 10 patients showed laminar flow, no turbulence/gradient at the inferior vena cava and mid-tunnel levels. The diameter indexed to body surface area showed growth, reduction, or no change depending on flow demands. The construction of the extracardiac lateral tunnel Fontan conduit using viable pedicled pericardium is a relatively simple, durable, and safe operation. Long-term follow-up confirms low morbidity and mortality. Fenestration is unnecessary in most patients. This viable tunnel adapts to physiologic flow demands.

The Modified Fontan Procedure and Prolonged Pleural Effusions

The modified Fontan procedure may be complicated by prolonged pleural drainage. Predisposing factors are not fully understood. This study examines perioperative variables associated with prolonged effusions. We examined the Fontan procedure in 41 patients. Mean age was 45 months (range, 9 to 113 months). Mean weight was 14.7 kg (range, 6.9 to 30.4 kg). Diagnoses included tricuspid atresia in 29 per cent, pulmonary atresia in 12 per cent, and double-outlet right ventricle in 12 per cent. Fontan revisions were excluded. The extracardiac Fontan was performed in 22 patients (54%) and the lateral tunnel (LT) in 18 (44%). Fenestration was performed in 56 per cent. Ten patients (24%) had prolonged pleural effusions. Mean chest tube output (24.2 vs 14.1 mL/kg/d, P < 0.01), days with chest tubes (10.2 vs 5.8 days, P < 0.01), and length of stay (20.8 vs 8.13 days, P < 0.01) were significantly greater in those with effusions. Preoperative mean pulmonary artery pressure was higher in patients with prolonged effusions (12.7 vs 9.90 mmHg, P = 0.001). No other factors were identified as risk factors. Prolonged pleural effusions (greater than 14 days) are common after the modified Fontan procedure. A higher preoperative mean pulmonary artery pressure may be predictive of these effusions. Fenestration, type of Fontan reconstruction, and size of extracardiac conduit did not predispose to postoperative effusions.

A DRAMATIC PRESENTATION OF FONTAN PROCEDURE COMPLICATIONS

INTRODUCTION: The Fontan procedure is an anatomically complex cardiac revision performed in children with univentricular congenital heart diseases, including tricuspid atresia. The surgery has undergone multiple revisions since its inception by Dr. Francis Fontan in 1971, with a resulting evolution in its complication profile. We present the case of a 23-year-old female who presents with a well-known and life-threatening complication of the Fontan procedure.

Trisomy 9 mosaicism and XX sex reversal

We report on a case of a phenotypic male infant who was referred to the genetics service due to dysmorphic features and congenital cardiac anomalies. Dysmorphic features included low-set, posteriorly rotated ears with squared, simple helices, midface hypoplasia, a broad nasal root with wide nasal alae, small mouth, micrognathia, short neck, overlapping fingers, rocker-bottom feet, prominent heels, and hypoplastic toenails. The phallus was normal and no testes were palpable in the scrotal sac. Cardiac anomalies included tricuspid atresia and a ventricular septal defect. The karyotype was 46,XX. Diagnostic microarray demonstrated evidence for trisomy 9 mosaicism, metaphase FISH revealed trisomy 9 on 7% of cultured cells, and interphase FISH analysis of a peripheral blood smear showed trisomy 9 in 78% of the cells examined. This is the first reported case of XX sex reversal in a patient with trisomy 9 mosaicism.

Successful Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula in a Newborn with Single Ventricle Physiology

A neonate with VACTERL association including tricuspid atresia was scheduled for thoracoscopic esophageal atresia with tracheoesophageal fistula (EA/TEF) repair and laparoscopic gastrostomy tube placement. In addition to standard noninvasive monitoring, arterial blood pressure, central venous pressure, and cerebral oxygen saturation were monitored. Gastric distension resulting from positive pressure ventilation prevented laparoscopic gastrostomy tube placement. Thoracoscopy with a CO2 insufflation pressure of 6 mm Hg at low flow (1 L/min) was well tolerated hemodynamically despite hypercarbia and cerebral oxygen saturation was maintained. Careful monitoring and good communication were critical to the safe management of this single ventricle patient during thoracoscopic EA/TEF repair. Esophageal and tracheoesophageal fistula in conjunction with single ventricle physiology carries a significant risk of mortality. We present the anesthetic management of a neonate with unpalliated tricuspid atresia who underwent thoracoscopic tracheoesophageal fistula repair.

The modified fontan procedure: early and late results in 132 adult patients

Objective The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient. Methods Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31. Results Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extra-cardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up. Conclusions In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life.

The Modified Blalock-Taussig Shunt: A 6-Year Experience from a Developing Country

Since 1992 we have performed the modified Blalock-Taussig shunt (MBTS) for cyanotic children in Libya. This retrospective study reviews our results as a developing country, comparing them with those in the literature, and makes suggestions to improve our results. Between May 1992 and May 1998, 94 children (58 males and 36 females) underwent 100 MBTSs in Mesallata Cardiothoracic Centre, Libya. The age ranged from 4 days to 15 years, (median 12 months) and 25 patients were neonates. Patients' weights ranged from 3 to 31 kg (median 6.4 kg). Eighty-nine shunts were performed on the left side and 11 on the right. A 6-mm polytetrafluoroethylene graft was used in 68 children and a 4-mm graft in 32 cases. Tetralogy of Fallot (TOF) comprised the majority of cases (63; 67%), the remaining 31 (33%) included tricuspid atresia (13), pulmonary atresia (9), univentricular heart complex (6), and others (3). Acute shunt failure occurred in 3 cases (2 with 4-mm and 1 with 6-mm grafts), all of which had not received perioperative heparin. Hospital mortality was 6% (6 patients); risk factors were neonates, a diagnosis other than TOF, and emergency surgery. There were 12 late deaths, 6 of which were due to sepsis. Follow-up was achieved in 82 of 88 early survivors for a period of 2 to 60 months (median 26 months). All surviving patients had subjective and objective improvement. We conclude that MBTS is an excellent palliative procedure for children requiring a systemic-pulmonary shunt in developing as well as developed countries. This series shows an early mortality rate comparable to that of other studies, but the late mortality is higher, mainly due to sepsis, which warrants further attention.

Predicting the identity of decompressing veins after cavopulmonary anastomoses

D veins are an important cause of unanticipated cyanosis after cavopulmonary anastomosis (CPA). Venous connections between the superior and inferior vena caval pathways result in cyanosis after bidirectional CPA (hemi-Fontan and bidirectional Glenn), whereas connections between the systemic and pulmonary venous pathways lead to cyanosis after either bidirectional CPA or total cavopulmonary connection (Fontan operation). Previous studies have focused on hemodynamic settings in which decompressing channels are likely to occur after bidirectional CPA. However the spectrum of anatomic variations resulting in significant venous decompression after total as well as superior cavopulmonary connections has not been well characterized. In this study, we defined the anatomy of significant decompressing veins occurring after CPA, determined the incidence of significant decompressing veins by reviewing angiograms in all patients after CPA, and determined whether cardiac anatomy or clinical parameters were predictive of decompressing venous anatomy. The cardiology database from The Children’s Hospital of Philadelphia was reviewed to identify patients with CPAs who had undergone cardiac catheterization between January 1987 and January 1998. Patients whose angiograms could be obtained and were of adequate quality for interpretation were included in the analysis. Echocardiographic diagnoses were reviewed. The anatomy of the underlying heart defect was classified into 1 of 4 categories: (1) hypoplastic left heart syndrome, (2) other functional single right ventricle (including double-outlet right ventricle), (3) functional single left ventricle (including tricuspid atresia and pulmonary atresia), and (4) heterotaxy syndrome. Hemodynamic information including arterial oxygen saturation, pulmonary artery pressures, and ventricular end-diastolic pressures was recorded. Angiograms were reviewed noting anatomy and size of decompressing veins. Magnification correction was based on the known diameter of the angiographic catheter. Decompressing vessels were deemed significant if they were 3 mm in diameter or associated with arterial oxygen saturation 1 SD below the mean for each group (superior or total CPA). In many patients, right azygos veins were intentionally left patent by the surgeon at the time of bidirectional CPA. The significance of decompressing right azygos veins was determined solely by saturation criteria. Finally, in patients with other sources of arterial desaturation after total cavopulmonary connection (baffle leak, fenestration, or partial hepatic vein exclusion), only size was considered in determining the significance of decompressing veins. Differences between groups were evaluated by Student’s t test or Wilcoxon rank-sum test for numerical data, and chi-square distribution for categorical variables. A p value 0.05 was considered statistically significant. Five hundred fifty patients were identified with CPA. Angiograms adequate for review were obtained for 298 of these patients. Hypoplastic left heart syndrome was present in 170 patients (47%), single left ventricle in 101 (28%), single right ventricle in 60 (17%), and heterotaxy syndrome in 32 (8%). Data were collected on 208 patients who had undergone hemi-Fontan or bidirectional Glenn procedures. The median age at catheterization was 19 months (range 4 months to 17 years). Thirty-three of these patients (16%) had significant decompressing veins. Pulmonary artery pressures were higher, and (partially by definition) arterial oxygen saturations were lower in patients with decompressing veins (p 0.05; Table 1). Four types of decompressing veins occurred after bidirectional CPA. Most common were azygos connections; 19 cases accounted for 58% of the total. This type included azygos veins to either the right or left superior vena cava (SVC), hemiazygos veins, and accessory hemiazygos veins (Figure 1A). Left SVC decompressing to a coronary sinus occurred in 8 paFrom the Department of Pediatrics, University of Pennsylvania School of Medicine and Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania. Dr. Rome’s address is: Division of Cardiology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. E-mail: rome@email.chop.edu. Manuscript received February 19, 2001; revised manuscript received and accepted July 23, 2001. TABLE 1 Hemodynamic Data from Patients With Bidirectional Cavopulmonary Anastomosis

Extracardiac conduit fontan procedure: early and intermediate results.

The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

Long-term follow-up of surgical patients with single-ventricle physiology: prognostic anatomical determinants

Although univentricular or biventricular repair is not always possible for hearts with single-ventricle physiology, it has been proven to produce a satisfactory outcome. It is hypothesized that patient prognosis can be predicted by anatomical diagnosis at presentation. Between 1961 and 1995, 158 patients with single-ventricle physiology, including tricuspid atresia and hypoplastic left heart syndrome, were referred to the authors' institute, and underwent 260 surgical interventions. Follow-up was 99% complete. Patient survival and anatomical risk factors were examined by the Kaplan–Meier method, and multivariate analysis including the Cox proportional hazard model. Mean (s.e.m.) actuarial survival rates at 1, 5, 10 and 20 years following birth were 70.3(3.6)%, 56.3(4.0)%, 48.8(4.2)%, and 40.9(4.7)%, respectively. Definitive palliation was attempted in 38 patients (univentricular in 35 and biventricular in three). Multivariate analysis identified systemic ventricular outflow tract obstruction, mitral atresia, situs ambiguus, and pulmonary vein drainage tract obstruction as independent prognostic factors for overall survival. Visceral heterotaxy was the only independent risk factor for lack of application or failure (death or take-down within 30 days of operation) of univentricular or biventricular repair. In conclusions, these anatomical factors in hearts with single-ventricle physiology affect long-term mortality, despite multiple and heterogeneous surgical efforts.