When should pulmonary artery angioplasty be performed for fontan candidates with pulmonary coarctation?

We performed concomitant pulmonary artery angioplasty and the Blalock-Taussig shunt under median sternotomy and cardiopulmonary bypass at the neonatal stage in Fontan candidates with pulmonary coarctation to obtain optimum pulmonary circulation. This surgical strategy realized appropriate early growth of the pulmonary artery necessary for the Fontan operation.     

Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

Pulmonary artery architecture and symmetry after palliative operations for hypoplastic left heart syndrome may affect subsequent suitability for a modified Fontan operation. Two-dimensional echocardiography was used to measure pulmonary artery diameter and assess symmetry after two types of systemic-pulmonary artery shunts: modified right Blalock-Taussig shunt (14 patients) and central shunt (from underside of aortic arch gusset to pulmonary artery confluence) (14 patients). Age, weight, preoperative diameter of right and left pulmonary arteries (proximal, middle, and distal segments), and mean interval between preoperative and postoperative echocardiographic studies (20.2 +/- 4.4 days in the Blalock shunt group; 19.1 +/- 6.8 days in the central shunt group) were similar. Early postoperatively, patients with a Blalock shunt showed a significant decrease in the diameter of all pulmonary artery segments except the distal right pulmonary artery. The diameters tapered from distal right to distal left pulmonary artery in this group. Patients with the central shunt had a significant decrease in the diameter of all pulmonary artery segments. There were no significant differences when cross comparisons were made of the various pulmonary arterial segments in patients after a central shunt. Similar findings persisted in 19 patients from both groups who had a late postoperative echocardiogram (mean interval between studies = 271 days in the group of 10 patients with Blalock shunt and 167 days in the group of nine patients with a central shunt). In conclusion, the central shunt preserves pulmonary artery symmetry, which may be important in candidates for the Fontan operation in infancy.     

Extracardiac Fontan operation without cardiopulmonary bypass

AIM: The avoidance of cardiopulmonary bypass and aortic cross-clamping in patients possessing single ventricular physiology has potential advantages including preservation of ventricular and pulmonary functions; early extubation, decreased incidence of pleural effusions and decreased requirement of inotropic agents and blood products. In this study, we assessed the postoperative outcome of patients who have undergone extracardiac Fontan operation performed without cardiopulmonary bypass. METHODS: Between March 1999 and August 2002, 10 consecutive patients (6 males and 4 females) underwent extracardiac Fontan operation without cardiopulmonary bypass. The age of patients ranged between 1.5 to 12 (5.2+/-3.1) years. All the patients requiring any intracardiac intervention were excluded from the study. Previous operations of the patients were modified Blalock-Taussig shunt procedure in 3 patients, bidirectional cavopulmonary shunt operation in 2 patients and pulmonary arterial banding in 1 patient. All operations were performed without cardiopulmonary bypass. Bidirectional cavopulmonary anastomosis was performed by using a transient external shunt constructed between the superior vena cava and right atrium. An appropriate sized tube graft was anastomosed to the inferior surface of right pulmonary artery. Finally, inferior vena cava to tube grafts anastomosis was performed with the aid of another external shunt constructed between inferior vena cava and right atrium. During the procedure central venous pressure, blood pressure and arterial oxygen saturation levels were continuously monitored and recorded. RESULTS: The mean intraoperative Fontan pressure was 16.1+/-2.75 mmHg. Intraoperative fenestration was required in 4 patients with a Fontan pressure above 18 mmHg. There were no intra and postoperative deaths. Three patients required mild doses of inotropic support during the postoperative period. All patients were weaned off mechanical ventilation within 24 h. The mean arterial oxygen saturation raised from 74.5+/-4.2% to 93.5+/-2%. Arterial oxygen saturation was 95+/-0.6% in 6 patients without fenestration and 91.2+/-0.5% in 4 patients with fenestration (P=0.001). All patients were in sinus rhythm postoperatively. Only 2 patients required blood transfusion. Two patients suffered from prolonged pleural effusion (more than 7 days). The mean intensive care unit and hospital stay periods were 3.3+/-1.5 and 15.4+/-5.3 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed without cardiopulmonary bypass provides good results in short and midterm follow-up periods with improved postoperative hemodynamics.     

As originally published in 1996. Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan patients. Updated in 2002

BACKGROUND: A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation. METHODS: Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 +/- 257 days). RESULTS: Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p = 0.01). Pumonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly from 1.8 +/- 1.1 to 0.7 +/- 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them. CONCLUSIONS: The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.     

One-stage unifocalization followed by staged Fontan operation

We report a successful case of staged Fontan operation for single ventricle, pulmonary atresia, major aortopulmonary collaterals and absent central pulmonary artery. The one-stage unifocalization, concomitant with central pulmonary artery creation by autologous pericardium and modified Blalock-Taussig shunt at 1 month of age as the first palliation, and the Glenn operation and pulmonary arterial augmentation with superior vena caval patch at 9 months of age as the second palliation were performed. Extracardiac conduit Fontan operation was completed at 22 months of age. The pulmonary artery continued developing with increasing pulmonary arterial index and stable pulmonary arterial pressure throughout the clinical course. The one-stage unifocalization in early infancy and the repeated surgical intervention to the pulmonary artery without prosthetic material provided excellent pulmonary arterial growth and Fontan completion.     

Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts.

The first superior vena cava-pulmonary artery shunt (Glenn shunt) in our series was performed in February 1958. From then through September 1988, 91 patients have undergone this procedure for a wide variety of congenital defects. We here report follow-up data available on all patients. Ages ranged from 2 days to 46 years (mean 6.8). Diagnoses were as follows: tricuspid atresia, 27; single ventricle, 22; tetralogy of Fallot, 14; D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, 9; D-transposition, 5; Ebstein's anomaly, 4; pulmonary atresia + intact septum, 4; and others, 6. The hospital mortality rate was 7.7% (one death in the last 53 patients, 1.9%). Five deaths occurred in patients less than 6 months old. There were 20 late deaths (22%) with actuarial survival rates of 84% and 66% at 10 and 20 years, respectively. Pulmonary arteriovenous fistula formation was seen in 18 patients (19.7%), six of whom have undergone therapeutic embolization with improvement in saturation. The prevalence of pulmonary arteriovenous fistula increases with time after shunt. No long-term shunt thrombosis or stricture formation was seen. Fifty percent of shunts were still functioning at 20 years. Palliation was limited because of decrease in blood flow to the contralateral pulmonary artery, collaterals between the inferior and superior venae cavae, and pulmonary arteriovenous fistula formation. Improvement in saturation was obtained in eight otherwise inoperable patients by creation of a right axillary arteriovenous fistula up to 19 years after the Glenn shunt. Three patients had conversion of a Blalock-Taussig shunt to a Glenn shunt with improvement in congestive heart failure. Twenty-six patients have undergone a Fontan procedure with two deaths. Compared with the group having a Fontan procedure without a prior Glenn operation, there was no difference in early or late mortality. Thirty years after a Glenn shunt, the first patient in this series is working full time after having undergone a modified Fontan procedure in 1981. We conclude that the Glenn connection, usually with supplemental procedures to enhance oxygenation, has provided excellent physiologic palliation with low mortality up to 30 years with no late thrombosis or stricture formation. The incidence of pulmonary arteriovenous fistula increases with time and can be effectively treated with embolization. Physiologic repair after the Glenn shunt carries a low mortality. Although currently used infrequently, superior vena cava-pulmonary artery shunting remains a useful method of palliation in selected patients.(ABSTRACT TRUNCATED AT 400 WORDS)     

The role of a staged approach for high-risk Fontan candidates.

The preoperative risk characteristics and surgical outcome were reviewed in 24 patients who underwent a one-stage Fontan procedure (One-stage Group) and in 16 patients who underwent bidirectional cavopulmonary shunt as an interim procedure prior to a subsequent Fontan procedure (Two-stage Group) to clarify the role of the staged approach for high-risk Fontan candidates. There were 2 hospital deaths after the one-stage Fontan, and another 2 after the takedown of the fenestrated Fontan to BCPS in patients considered to be less than ideal candidate because of the presence of 3 or more risk factors. In contrast, among the total of 16 patients in the Two-stage Group who had significantly more risk factors than those in One-stage Group (2.8 +/- 1.4, vs 1.1 +/- 1.2 p < 0.001), 14 patients survived after bidirectional cavopulmonary shunt, and a subsequent Fontan procedure was accomplished in 12 patients of these 14, with 2 operative deaths after the takedown to bidirectional cavopulmonary shunt. The rate of final Fontan completion in the Two-stage Group (10/16, 62.5%) was considered to be reasonable, considering the fact that this patient group essentially includes non-Fontan candidates. Additionally, in the category of high-risk with 3 or more risk factors, the rate of successful Fontan completion was higher in the Two-stage Group than in the One-stage Group (50% vs 25%). In the survivors of the Two-stage Group, the average number of risk factor decreased from 2.7 +/- 1.3 (range 0 to 5) to 1.0 +/- 1.0 (range 0 to 4) after bidirectional cavopulmonary shunt with concomitant procedures (i.e., extended pulmonary artery reconstruction in 8, and repair of atrioventricular valve regurgitation in 4), predominantly due to improved Fontan candidacy, relating to pulmonary and/or ventricular characteristics, and eliminated anatomical risks. In conclusion, the two-stage approach with bidirectional cavopulmonary shunt accompanied by concomitant repair of associated anomaly may be useful to lower the risk enough to allow subsequent Fontan completion in high-risk patients.     

Pediatric heart transplantation after operations involving the pulmonary arteries

A prohibitive perioperative mortality has been previously ascribed to pediatric heart transplantation after palliative operations for congenital heart disease involving the pulmonary arteries. Of 46 children who have undergone heart transplantation at our institution between June 1984 and February 1990, 7 (15%; mean age 8 +/- 3 years; range 1 to 18 years) have previously undergone such operations: right ventricle to pulmonary artery conduit/homograft for levo-transposition of the great arteries (2), Waterston shunt for tricuspid and pulmonary atresia (1), pulmonary artery banding for single ventricle (1), Fontan procedure for single ventricle (1), first-stage Norwood procedure for hypoplastic left heart syndrome (1), and classic right Blalock-Taussig shunt for atrioventricular canal with pulmonic stenosis (1). Three categories of pulmonary artery anatomy that require different approaches to reconstruction at the time of transplantation are recognized: abnormalities of position, pulmonary outflow obstruction, and previous systemic- or atrial-pulmonary connections. At operation, individualized pulmonary arterial reconstruction was employed, including use of previously created right ventricular-pulmonary artery conduits/homografts and angioplasty (with and without pericardial patches). Transplantation was successful in all patients. Posttransplant right ventricular-pulmonary artery pressure gradients and pulmonary vascular resistance indices were acceptable, with a tendency to decrease with time. Two patients had critical right ventricular failure postoperatively; one of them required support with extracorporeal membrane oxygenation. There was no perioperative mortality, with three deaths occurring from 5 to 39 months after transplantation. All surviving patients are in New York Heart Association functional class I. Techniques borrowed from the repair of congenital cardiac lesions can be applied to subgroups of children undergoing heart transplantation. Additional length of donor aorta and pulmonary artery should be harvested for possible use in designing pulmonary artery connections. Previous palliative operations involving the pulmonary arteries with associated complex pulmonary artery anatomy are not of themselves an insurmountable obstacle to successful heart transplantation.     

The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt

BACKGROUND: The infant with a single ventricle and excessive pulmonary blood flow requires early protection of the pulmonary vascular bed to insure suitability for a subsequent Fontan procedure. The traditional approach, pulmonary artery banding, has had disappointing results. We have pursued an alternate strategy: division of the pulmonary artery, and placement of a systemic-to-pulmonary artery shunt. Potential sites of systemic outflow tract obstruction are simultaneously bypassed, by either a Damus-Kaye-Stansel, or modified Norwood procedure. METHODS: From January 1996 to June 2001, 22 infants were treated by this strategy. Patients with hypoplastic left heart syndrome were excluded. Median age was 18 days (range 2 days to 6 months). In addition to pulmonary artery division and shunt, 3 of 22 patients underwent a Damus-Kaye-Stansel procedure, and 13 of 22 patients underwent a modified Norwood procedure. RESULTS: There were no operative deaths, and one late death. Actuarial survival beyond 30 months was 90%. At follow-up catheterization in 22 patients, median transpulmonary gradient was 7 mmHg (range 4 to 18), and median pulmonary vascular resistance 1.9 Wood units (range 0.9 to 3.3). Twenty-one patients have undergone a subsequent bidirectional superior cavopulmonary connection, and 6 a Fontan procedure, with no deaths. No patient developed subaortic stenosis, or aortic arch obstruction. Neoaortic insufficiency was none or trivial in 12 patients, mild in 3, and moderate in 1. CONCLUSIONS: In patients with a functional single ventricle and excessive pulmonary flow, a strategy of pulmonary artery division and shunt, along with prophylactic bypass of systemic outflow obstruction, carries low operative and midterm mortality. It provides consistent protection of the pulmonary vascular bed, avoids subaortic stenosis and aortic arch obstruction, minimizes neoaortic insufficiency, and ensures suitability for progression along a Fontan pathway. These results provide a comparison for alternate strategies, including pulmonary artery banding.     

Effects of additional pulmonary blood flow after bidirectional Glenn procedure

Thirteen cases of functional single ventricle who had undergone bidirectional Glenn procedure were divided into 2 groups according to presence (5) or absence (8) of additional pulmonary blood flow. Additional flow was preserved in cases with relatively small pulmonary artery index (PA index), and their sources were antegrade pulmonary blood flow (2), and Blalock-Taussig (BT) shunt (3). In the control group, PA index was reduced to about 70% of the preoperative value, while in the additional group, pulmonary artery growth was recognized without significant elevation of mean pulmonary artery pressure. However, atrioventricular valve regurgitation progressed and systemic ventricular volume did not decrease after Glenn in the additional group. Therefore special consideration for the timing of Fontan procedure is mandatory.     

Surgical treatments of nonconfluent pulmonary arteries with congenital cardiac defects]

Pulmonary artery angioplasty or reconstruction was performed in seven patients with nonconfluent pulmonary arteries and congenital cardiac defects. Age of these patients were ranged from 6 months to 41 years old. Five of them had pulmonary truncal atresia and complex cardiac anomalies. Two of these five patients demonstrated nonconfluent pulmonary arteries due to deformities at ductal insertion of pulmonary arteries. Three patients had had previous systemic to pulmonary artery shunt operations which caused pulmonary artery distortions. Other two patients had intrapulmonary arterial obstructions due to pulmonary artery thrombosis. Patch pulmonary artery plasty was carried out in three patients, dilatation of severe stenotic pulmonary artery was done in one patient simultaneously with pulmonary valvotomy. Central shunt operation was added in one patient with the pulmonary artery which was unable to be reconstructed. Last two patients underwent intrapulmonary artery reconstruction with the rolled pericardial graft. Hospital death occurred in one patient with unproperly increased pulmonary blood flow by central shunt. Average follow-up period of these six survivors after operation was 1.4 +/- 0.8 years. As definite repairs, two patients had Fontan operation, two patients had right ventricle to pulmonary artery reconstruction. And remaining two patients are still to be followed until sufficient growth of pulmonary artery suitable for Fontan operation.     

The bidirectional cavopulmonary shunt.

The bidirectional cavopulmonary shunt improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Since 1983, 17 patients have undergone a cavopulmonary shunt procedure (five primary operations, 12 secondary operations). Diagnoses were single ventricle complex (n = 4), hypoplastic right heart syndrome (n = 10), and hypoplastic left ventricle (n = 3). Age at primary operation ranged from 3 1/2 to 30 months (median 6 months). Weight ranged from 3.5 to 9.7 kg. Age at secondary operation ranged from 10 months to 14 years (median 15 months). Seven cavopulmonary shunt operations were performed without cardiopulmonary bypass (six via thoracotomy and one via sternotomy) and 10, with cardiopulmonary bypass. All patients in the bypass group had additional procedures: takedown of modified Blalock-Taussig shunt, seven patients; revision of right ventricular outflow tract, four patients; reconstruction of pulmonary arteries, four patients; tricuspid valvuloplasty, one patient; and Damus procedure, one patient. There was one (1/17) operative death (Damus procedure). One patient required early revision. Follow-up ranges from 1 to 53 months (median 23 months). Twelve of 16 had a good to excellent late result, with a rise in mean arterial oxygen saturation from 69% to 83%. Three patients died late (4 to 53 months) (pulmonary vascular disease, pulmonary arteriovenous malformations, and pneumonia, one patient each). There was one late failure (converted to Glenn shunt). The cavopulmonary shunt is an excellent palliative procedure when right atrium-pulmonary artery connection (modified Fontan) must be deferred because of age, weight, or anatomic considerations. Five patients have undergone right atrium-pulmonary artery connection later. In addition, at the time of the modified Fontan operation, the cavopulmonary shunt approach may optimize the anatomic connection (eight additional patients).     

Modified Fontan operation for a single ventricle with a nonconfluent pulmonary artery]

A 8-year-old boy with a double inlet right ventricle with a non-confluent pulmonary artery was operated on with a modified Fontan operation. He had right isomerism, right aortic arch, bilateral superior caval veins, and left-sided inferior caval vein. Hepatic veins were separately drained to the right-side atrium. Left Blalock-Taussig shunt and right central shunt operations had been previously performed. Firstly, we had reconstructed the central pulmonary artery with a 16 mm porcine pericardial roll to unify the nonconfluent pulmonary arteries. Secondly, about 2 months after the first operation, we performed a modified Fontan operation. Systemic venous return from the inferior caval vein and the hepatic veins were drained to a reconstructed pericardial roll with an intraatrial Gore-Tex graft, and bilateral superior caval veins were also anastomosed to the roll. The structure of the pulmonary arterial system is one of the most important factors to determine the outcome of a modified Fontan operation. Even if the central pulmonary artery is absent, however, a modified Fontan operation is applicable for the patient whose peripheral pulmonary arteries have enough growth.     

Growth of the pulmonary arteries after systemic-pulmonary shunt.

Pulmonary artery growth after a systemic-pulmonary shunt was angiographically evaluated in 19 out of 35 patients. The mean age of the subjects at the time of the initial operation was 18+/-18 months including 12 patients under a year old. The preoperative diagnosis was tetralogy of Fallot (TOF) in 10 patients, TOF plus pulmonary atresia in five and transposition of great arteries in four. A Blalock-Taussig shunt (BTS) operation was performed in 16 patients (15 classical and 1 modified) and a central shunt was performed in three patients as an initial operation. The preoperative pulmonary artery index (PAI) was 129+/-42 in all patients and there were no significant differences between patients under or over a year old (139+/-42 vs. 115+/-49). Postoperative angiography was performed 32+/-13 months after the surgery. Room air arterial O2 pressure increased significantly from 29+/-5 mmHg to 42+/-5 mmHg just after an initial palliative shunt operation. PAI change in patients under a year old was 214+/-73%, which was higher than 145+/-27% in patients over a year old after a palliative shunt operation. On the ipsilateral side, PAI change was almost the same between patients under and over a year old. On the contralateral side, PAI change in patients under a year old was 216+/-68%, which was significantly higher than the 116+/-21% in patients over one year old. There was a significant negative correlation (r=-0.65, p<0.05) between PAI change and arterial O2 pressure as measured just after a palliative shunt operation. In conclusion, a palliative shunt operation prior to a year old is desirable in order to produce sufficient and bilateral pulmonary artery growth.     

Does the modified Blalock-Taussig shunt cause growth of the contralateral pulmonary artery?

BACKGROUND: Although some pediatric cardiology departments have a policy of adopting primary correction of tetralogy of Fallot in all symptomatic infants, we and others still palliate neonates and infants. Effective palliation should ameliorate symptoms and allow growth of the pulmonary arteries. Although studies on the growth of the ipsilateral and contralateral pulmonary arteries after a classic Blalock-Taussig shunt have been reported, pulmonary artery growth after a modified Blalock-Taussig shunt has not been studied as thoroughly. Therefore, we examined whether there is equal growth of the contralateral pulmonary artery after a modified Blalock-Taussig shunt. METHODS: We retrospectively analyzed the records of 140 patients with symptomatic tetralogy of Fallot who had a modified Blalock-Taussig shunt between October 1985 and October 1995. The median age at the time of the Blalock-Taussig shunt was 1.6 months. All patients had corrective procedures at a median age of 1.7 years. Cineangiography was done before the corrective procedure. From the angiograms the diameter of the right and left pulmonary arteries before their first lobar branches and the diameter of the descending thoracic aorta at the level of the diaphragm were measured. For each patient the ratios of right pulmonary artery to descending thoracic aorta and left pulmonary artery to descending thoracic aorta were determined and compared using Student's t test. RESULTS: Of the 140 patients, 114 had a left-sided Blalock-Taussig shunt, 20 had a right-sided shunt, and 6 patients had bilateral shunts. The mean right pulmonary artery to descending thoracic aorta ratio was 1.10 and the mean left pulmonary artery to descending thoracic aorta ratio was 0.98. This difference was not significant. CONCLUSION: We showed equal growth of the right and left pulmonary arteries with no distortion after a modified Blalock-Taussig shunt. If palliation is considered, the modified Blalock-Taussig shunt remains our choice.     

A case for anatomic correction in atrioventricular discordance? Effects of surgery on tricuspid valve function

OBJECTIVE: To assess tricuspid valve function in atrioventricular discordance after palliative procedures (pulmonary artery banding and Blalock-Taussig shunt) and corrective procedures (anatomic and physiologic repair). METHODS: Tricuspid valve dysfunction was assessed by transthoracic echocardiography and graded as no regurgitation (0), mild (1), moderate (2), and severe (3) before and after palliative and corrective procedures performed in 97 patients with atrioventricular discordance between 1988 and 1999. Thirty-two percent had an isolated ventricular septal defect, 43% had a ventricular septal defect and pulmonary stenosis, and 16% had pulmonary stenosis. Twenty-six patients underwent pulmonary artery banding and 28 had a Blalock-Taussig shunt. Seventy patients underwent physiologic and 19 underwent anatomic repair. Six patients underwent one-ventricle repair. RESULTS: After pulmonary artery banding, the tricuspid regurgitation score decreased from 1.7 +/- 0.8 to 0.9 +/- 0.6 (P <.001). In patients who underwent a Blalock-Taussig shunt, the tricuspid regurgitation score increased from 0.7 +/- 0.5 preoperatively to 1.4 +/- 0.6 postoperatively (P <.001). After physiologic repair, there was no significant change in the tricuspid regurgitation score; however, 7 patients required additional repair or replacement. The regurgitation score was significantly reduced from 1.5 +/- 0.8 to 0.4 +/- 0.5 (P <.001) after anatomic repair. The operative mortality in patients who underwent physiologic repair was 7% as compared with 0% in the anatomic repair group (P =.59). The median follow-up was 3.2 years. CONCLUSIONS: Right ventricular volume loading (shunt) worsens tricuspid regurgitation, whereas volume reduction (banding) or left-to-right septal shift (anatomic repair) has beneficial effects. We have not observed a significant change in the tricuspid regurgitation score after physiologic repair. Anatomic repair can be performed in selected patients with atrioventricular discordance and provides superior functional results.     

Pulmonary Atresia with Intact Ventricular Septum: Surgical Management Based on Right Ventricular Infundibulum

Abstract The optimal management of infants with pulmonary atresia with intact ventricular septum (PA.IVS) remains a controversy. Attempts have been made to base the surgical approach on various geometrical or morphological characteristics of the right ventricle (RV). However, the overall results remain poor when compared to other complex congenital heart defects. Forty-eight neonates with PA.IVS were admitted to our unit between 1980 and 1992. The management plan has evolved to be based entirely on the echocardiographic assessment of the state of development of the infundibulum of the RV. In neonates with a well-formed infundibulum (n = 31), the initial palliation consisted mainly of pulmonary valvotomy (without cardiopulmonary bypass) and PTFE shunt from the left subclavian artery to the main pulmonary artery. There was one death from initial palliation in this subgroup. If necessary, the RV cavity was later enlarged by excision of the hypertrophic muscle of both the trabecular and infundibular portions, before finally attempting biventricular repair. The actuarial probability of achieving a biventricular repair at 40 months of age was 60% (95% CL = 39.5% to 71.3%). Thirteen patients have undergone biventricular repairs with one late death over a total follow-up of 1,720 patient months. In one patient, the RV failed to grow satisfactorily, necessitating a Fontan procedure. Seventeen patients without a well-formed infundibulum were approached with a Fontan procedure in mind. The initial palliation in these patients consisted of a modified Blalock-Taussig shunt only. Ten have undergone a Fontan procedure so far and five are awaiting such repairs. In this group there were four operative deaths: two after initial palliation, and two after Fontan procedures. In patients with a well-developed infundibulum, the actuarial survival probability was 93% (95% CL = 74% to 98%) at 8 months with no further late deaths over 120 months follow-up, whereas in patients without a well-formed infundibulum it was 75% at 40 months (95% CL = 46% to 89%). The overall survival probability at 104 months was 77% (95% CL = 51% to 90%).     

Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'?

OBJECTIVE: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the 'Ten Commandments', are still applicable in the current era. MATERIALS AND METHODS: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8-7.1 years). The single functional ventricle was of left (n=241, 59%) or right ventricular morphology (n=163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n=162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n=50, 12%) or extracardiac conduit (n=194, 48%). They were fenestrated in 216 patients (53%). RESULTS: The early mortality was 4.4% (n=18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p<0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p=0.83, p=0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90+/-2%, 86+/-2% and 82+/-3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83+/-4%, 76+/-4% and 74+/-8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. CONCLUSIONS: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.     

First-stage angioplasty for a single ventricle with pulmonary artery coarctation

We report first-stage palliation consisting of pulmonary artery angioplasty and a systemic-to-pulmonary shunt using a minimized cardiopulmonary bypass technique performed with modified ultrafiltration for two patients with a single ventricle, pulmonary atresia, patent ductus arteriosus, and pulmonary coarctation during early infancy. Postoperative early extubation (15 and 18 h) and bilateral pulmonary artery growth before the second stage (Nakata indexes, in mm²/m²: right 94, left 53; and right 209, left 70) were experienced. Less-invasive pulmonary artery continuity reconstruction at an early stage is advantageous for low-weight patients with a single ventricle and pulmonary coarctation.     

Pulmonary thromboembolism after one and a half ventricle repair

Acute pulmonary thromboembolism in a patient who had undergone bidirectional Glenn anastomosis was treated by percutaneous selective intravascular thrombolysis. A 20-year-old woman was diagnosed with pulmonary stenosis and right ventricular hypoplasia, complete occlusion of the left pulmonary artery secondary to a Blalock-Taussig shunt, and atrial septal defect. The patient developed thromboembolism of the subsegmental branches of the right pulmonary artery resulting in critical hemodynamic deterioration 2 weeks after undergoing one and a half ventricle repair (bidirectional Glenn shunt). The patient was treated with tissue plasminogen activator administered directly into the right pulmonary artery via an intravascular catheter. Progressive recanalization of the obstruction began immediately. Pulmonary angiography 3 months after thrombolytic therapy demonstrated patent subsegmental vessels. Early detection of the pulmonary thromboembolism and prompt intervention are crutial to relieving this fatal complication after a Fontan operation.