New developments in the treatment of hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) was once a uniformly fatal disease, accounting the majority of neonatal deaths due to congenital heart defects. Twenty-five years of advances in the surgical and medical management of this disease have resulted in dramatic improvements in survival for these children. The goal for patients with HLHS should be survival of near 100% with a good quality of life. The advances described in this manuscript detail some of the new techniques used in the surgical and medical management. For infants undergoing staged reconstruction, the Norwood procedure is performed in the newborn period, followed by a hemi-Fontan operation at 6 months of age, and a modified Fontan operation at 1 to 2 years of age. A significant recent modification of the Norwood procedure is the placement of a right ventricle to pulmonary artery (RV-PA) conduit instead of a systemic artery to pulmonary shunt (modified Blalock-Taussig or BT shunt) as the source of pulmonary blood flow. Our recent experience with this modification demonstrated an incremental increase in survival, improved postoperative stability, and decreased inter-stage mortality. At catheterization, significant differences in hemodynamic parameters were present that were consistent with improved coronary blood flow, decreased volume load to the single right ventricle, and improved pulmonary artery growth in those patients with the RV-PA conduit. The 3rd stage in the surgical treatment of HLHS is the modified Fontan operation or cavo-pulmonary anastamosis. A new approach for Fontan completion is the placement of a stent covered with a thin layer of Goretex from the inferior vena cava to the hemi-Fontan baffle. This can be performed in the catheterization laboratory, with a decrease in post-procedure pleural effusions, lower mortality, and a shorter length of stay. The future of treatment for HLHS may eventually involve a single open-heart procedure with initial and final interventions being performed in the catheterization laboratory. Some cardiac centers perform transplantation for management of HLHS. Survival following transplantation has improved as advances in the pre- and post-operative management continue, along with new options for immunosuppression. Treatment for HLHS continues to evolve, as ongoing work has resulted in improved short and long-term survival. The future for children with this disease is encouraging as evidenced by the remarkable achievements made to date and the current worldwide interest and study of HLHS.

Allopurinol neurocardiac protection trial in infants undergoing heart surgery using deep hypothermic circulatory arrest.

OBJECTIVE: This pharmacologic protection trial was conducted to test the hypothesis that allopurinol, a scavenger and inhibitor of oxygen free radical production, could reduce death, seizures, coma, and cardiac events in infants who underwent heart surgery using deep hypothermic circulatory arrest (DHCA). DESIGN: This was a single center, randomized, placebo-controlled, blinded trial of allopurinol in infant heart surgery using DHCA. Enrolled infants were stratified as having hypoplastic left heart syndrome (HLHS) and all other forms of congenital heart disease (non-HLHS). Drug was administered before, during, and after surgery. Adverse events and the clinical efficacy endpoints death, seizures, coma, and cardiac events were monitored until infants were discharged from the intensive care unit or 6 weeks, whichever came first. RESULTS: Between July 1992 and September 1997, 350 infants were enrolled and 348 subsequently randomized. A total of 318 infants (131 HLHS and 187 non-HLHS) underwent heart surgery using DHCA. There was a nonsignificant treatment effect for the primary efficacy endpoint analysis (death, seizures, and coma), which was consistent over the 2 strata. The addition of cardiac events to the primary endpoint resulted in a lack of consistency of treatment effect over strata, with the allopurinol treatment group experiencing fewer events (38% vs 60%) in the entire HLHS stratum, compared with the non-HLHS stratum (30% vs 27%). In HLHS surgical survivors, 40 of 47 (85%) allopurinol-treated infants did not experience any endpoint event, compared with 27 of 49 (55%) controls. There were fewer seizures-only and cardiac-only events in the allopurinol versus placebo groups. Allopurinol did not reduce efficacy endpoint events in non-HLHS infants. Treated and control infants did not differ in adverse events. CONCLUSIONS: Allopurinol provided significant neurocardiac protection in higher-risk HLHS infants who underwent cardiac surgery using DHCA. No benefits were demonstrated in lower risk, non-HLHS infants, and no significant adverse events were associated with allopurinol treatment.congenital heart defects, hypoplastic left heart syndrome, induced hypothermia, ischemia-reperfusion injury, neuroprotective agents, allopurinol, xanthine oxidase, free radicals, seizures, coma.

Reduction in levels of triiodothyronine following the first stage of the Norwood reconstruction for hypoplastic left heart syndrome.

OBJECTIVE: Thyroid hormone has important effects on cardiovascular performance. This study was performed to evaluate the changes in levels of triiodothyronine following the first stage of reconstruction for hypoplastic left heart syndrome. METHODS: We enrolled 14 newborns with hypoplastic left heart syndrome scheduled for first stage reconstruction. Blood samples were obtained pre-, intra-, and post-operatively. Levels of free and total triiodothyronine were determined by radioimmunoassay. Statistical comparison was performed using Wilcoxon's signed rank test. RESULTS: The levels of free triiodothyronine decreased from a baseline of 355+/-31 pg/dl to 205+/-21 pg/dl upon the institution of bypass, and declined to a level of 135+/-9 pg/dl at 24 hours postoperatively. Similarly, levels of total triiodothyronine decreased from 101+/-15 ng/dl to 65+/-4 ng/dl upon the institution of bypass, and continued to decline during the first 24 hours postoperatively. Levels of free and total triiodothyronine had returned to baseline by the fifth postoperative day. CONCLUSIONS: The data demonstrate significant decreases in levels of free and total triiodothyronine during the early postoperative period. These changes in levels of thyroid hormone may have adverse effects on cardiac function during this phase of recovery.

Is there a role for extracorporeal life support after stage I Norwood?

OBJECTIVES: Controversy still surrounds the use of extracorporeal life support (ECLS) in patients with single ventricle physiology. An analysis of the experience with a group of neonates who underwent stage I Norwood palliation and received ECLS is reported. METHODS: A retrospective review of hospital records, operative notes and perfusion data was performed in a group of 12 consecutive patients undergoing ECLS in the perioperative period after stage I. An analysis to identify risk factors for poor outcome was performed. RESULTS: The mean age and weight were 3.9 days (1-14) and 2.6 kg (1.4-3.8), respectively. There were 3/12 patients under 2.5 kg and four patients were under 35 weeks of gestation. The most common diagnosis (7/12) was hypoplastic left heart syndrome with aortic atresia. Associated extracardiac anomalies were present in four patients. Three patients had an additional cardiac defect and two of these required an additional surgical procedure besides the stage I Norwood. The mean circulatory arrest was 56 min (46-63). ECLS was initiated preoperatively in one, intraoperatively in nine, and postoperatively in two patients. The indication for support was: arrhythmia (one), low output (six), cardiac arrest (three), unbalanced circulation (one), and hypoxemia (two). The mean duration of support was 68 h (24-192). Eight patients were weaned off support, and six were discharged home in good condition. Morbidity included sepsis in five, renal failure in five, neurologic sequelae in three, and bleeding in two. Prematurity, renal dysfunction and the initiation of ECLS outside the operating room were significantly associated with poor outcome. Residual hemodynamic effect, low birth weight and neurologic event showed a tendency towards poor outcome, but did not reach statistical significance. CONCLUSIONS: Although the use of ECLS in patients with single ventricle physiology still carries a significant risk, prompt initiation of support can improve the outcome in a group of patients with impaired cardiopulmonary function after stage I palliation.

Preoperative risk-of-death prediction model in heart surgery with deep hypothermic circulatory arrest in the neonate.

OBJECTIVE: Our goal was to generate a preoperative risk-of-death prediction model in selected neonates with congenital heart disease undergoing surgery with deep hypothermic circulatory arrest. METHODS: We completed a single-center, prospective, randomized, double-blind, placebo- controlled neuroprotection trial in selected neonates with congenital heart disease requiring operations for which deep hypothermic circulatory arrest was used. An extensive database was generated that included preoperative, intraoperative, and postoperative variables. Variables (delivery, maternal, and infant related) were evaluated to produce a preoperative risk-of-death prediction model by means of logistic regression. An operative risk-of-death prediction model including duration of deep hypothermic circulatory arrest was also generated. RESULTS: Between July 1992 and September 1997, 350 (74%) of 473 eligible infants were enrolled with 318 undergoing deep hypothermic circulatory arrest. The mortality was 52 of 318 (16.4%), unaffected by investigational drug. The resulting preoperative risk model contained 4 variables: (1) cardiac anatomy (two-ventricle vs single ventricle surgery, with/without arch obstruction), (2) 1-minute Apgar score (5), (3) presence of genetic syndrome, and (4) age at hospital admission for surgery (5 days). Mortality for two-ventricle repair was 3.2% (4/130). Mortality for single ventricle palliation was 25.5% (48/188) and was significantly influenced by Apgar score, genetic diagnosis, and admission age. The preoperative model had a prediction accuracy of 80%. The operative risk model included duration of deep hypothermic circulatory arrest, which significantly (P =.03) increased risk of death, with a prediction accuracy of 82%. CONCLUSIONS: In this selected population, postoperative mortality risk is significantly affected by preoperative conditions. Identification of infants with varying mortality risks may affect family counseling, therapeutic intervention, and risk stratification for future study designs.

Tetralogy of Fallot with anomalous coronary artery: double outflow technique.

A new technique to repair tetralogy of Fallot with an anomalous coronary artery crossing the right ventricular outflow tract is described, together with intermediate term follow-up. Using a pedicled flap of the anterior pulmonary artery wall as the floor, and a vascular or prosthetic patch as the roof, a composite conduit with the potential for growth is constructed. Together with the native outflow tract, this provides unobstructed egress from the right ventricle to the branch pulmonary arteries. Since 1990, 4 infants aged 2-weeks to 6-months have undergone primary repair using this technique. Intermediate term follow-up shows adequate durability of the repair.

Hepatic venous malformation after modified Fontan procedure with partial hepatic vein exclusion.

Partial hepatic vein exclusion, as an alternative to baffle fenestration, has been performed as a modification of the Fontan procedure. We report a case of severe cyanosis resulting from massive right-to-left shunting via a hepatic vascular malformation that developed in a patient after a modified Fontan procedure with partial hepatic vein exclusion.

Early reduction of the volume work of the single ventricle: the hemi-Fontan operation.

BACKGROUND: In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume work associated with parallel pulmonary and systemic circulations. The hemi-Fontan operation was conceived as a means of accomplishing early reduction of the volume work of the single ventricle. METHODS: All patients presenting in infancy with single-ventricle physiology were managed by early hemi-Fontan operation in anticipation of a subsequent completion Fontan operation. Between May 1989 and August 1995, 400 patients less than 2 years of age underwent hemi-Fontan operations. Mean age at operation was 8.5 months (range, 2 months to 24 months). The hemi-Fontan operation included association of superior vena(e) cava(e) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the superior vena cava into the right atrium, and elimination of other sources of pulmonary blood flow. RESULTS: Operative mortality ( < 30 days) was 31 of 400 patients (7.8%). For the last 200 patients, operative mortality was 8 of 200 (4.0%). Younger age at operation was not an independent risk factor for operative mortality. Urgent operation in the presence of a hemodynamic burden requiring concomitant procedures was associated with increased mortality. CONCLUSIONS: The hemi-Fontan operation can be accomplished with low operative mortality in young patients, achieving early reduction of the volume work of the single-ventricle heart.

Doppler echocardiographic analysis of flow in the ductus arteriosus of infants with hypoplastic left heart syndrome: relationship of flow patterns to systemic oxygenation and size of interatrial communication.

Excessive pulmonary overcirculation related to imbalance in the pulmonary/systemic vascular resistance ratio contributes to hemodynamic instability in infants with the hypoplastic left heart syndrome. Because the ductus arteriosus bridges the two vascular circuits in this lesion, we studied the Doppler echocardiographic flow patterns in the ductus arteriosus of 50 infants with hypoplastic left heart syndrome to investigate their relationship to the degree of pulmonary blood flow as measured by simultaneously obtained levels of partial pressure of oxygen in arterial blood. The degree of restriction to pulmonary venous egress as determined by size of the interatrial communication was also correlated with ductal flow patterns and partial pressure of oxygen in arterial blood. Biphasic flow was noted in all infants. Mean peak velocity of antegrade flow (pulmonary artery to aorta) was greater than that of retrograde flow (aorta to pulmonary artery) (131 +/- 45 cm/sec versus 54 +/- 15 cm/sec; p < 0.001), mean time of retrograde flow was greater than that of antegrade flow (246 +/- 60 msec versus 174 +/- 28 msec; p < 0.001), and mean velocity-time integral of antegrade flow was greater than that of retrograde flow (13.3 +/- 4.8 cm versus 6.3 +/- 3.4 cm; p < 0.001). The ratio of velocity-time integral of retrograde flow/antegrade flow (a volumetric estimate of diastolic reversal into the pulmonary vascular bed indexed to systemic output) correlated extremely well with partial pressure of oxygen in arterial blood (r = 0.91; p < 0.0001). Categoric size of the interatrial communication (none, n = 2; small [<2 mm], n = 9; moderate [3 to 4 mm], n = 23; and large [>4 mm], n = 16) correlated with partial pressure of oxygen in arterial blood (r = 0.82; p < 0.001); the smaller the interatrial communication the lower the partial pressure of oxygen in arterial blood and velocity-time integral ratio of retrograde/antegrade flow. Doppler flow patterns in the ductus arteriosus of infants with hypoplastic left heart syndrome are reflective of the resistance ratio between the pulmonary and systemic vascular circuits and may be helpful in monitoring the hemodynamics of these infants.

Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration.

A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

Avoidance of subaortic obstruction in staged management of single ventricle.

BACKGROUND: Subaortic obstruction is a frequent accompaniment of single-ventricle anatomy. Most often, the aorta arises from an outflow chamber that is connected to the single ventricle by a bulboventricular foramen or ventricular septal defect. This connection may be restrictive of flow at birth, or may become obstructive after surgical procedures that reduce the volume work of the ventricle. Subaortic obstruction is recognized as a risk factor for reconstructive surgical procedures for single ventricle. METHODS: To prevent the consequences of subaortic obstruction, we have routinely amalgamated the proximal main pulmonary artery with the ascending aorta and arch early in the management of these patients. From September 1990 through September 1994, 29 neonates and infants with single ventricle and established or potential subaortic obstruction underwent staged reconstructive surgical procedures. The initial operation in the newborn period was a Norwood procedure (18 patients) or a pulmonary artery band (5 patients). All survivors underwent a hemi-Fontan procedure at approximately 6 months. RESULTS: Eighteen patients have undergone a completion Fontan operation with no deaths. Five await completion Fontan. None has subaortic obstruction, and none has pulmonary valve insufficiency that is graded more than mild. CONCLUSIONS: Early association of the proximal main pulmonary artery with the ascending aorta appears to obviate the risks and complications associated with subaortic obstruction in patients with single ventricle.

Acute changes in left ventricular geometry after volume reduction operation.

BACKGROUND: After surgical removal of a volume load, regression of myocardial mass proceeds slowly relative to diminution in ventricular cavity size, resulting in increased wall thickness and decreased cavity dimensions, which may affect the filling properties and performance of the heart. We investigated the acute changes in ventricular geometry that occur after the Fontan operation and hemi-Fontan operation for tricuspid atresia, and compared them with closure of a ventricular septal defect in a two-ventricle heart. METHODS: We reviewed the results of echocardiography performed before and 8 +/- 7 days after (1) Fontan operation for tricuspid atresia (n = 9), (2) hemi-Fontan operation for tricuspid atresia (n = 10), and (3) closure of a ventricular septal defect (n = 13). Measurements were made from images of the left ventricle at end-diastole: (1) apical, septal, and posterior wall thickness; and (2) long- and short-axis cavity diameters, cross-sectional areas, and ventricular volume. Posterior wall thickness to cavity dimension ratio was calculated. RESULTS: Wall thickness increased in all groups, with the greatest degree of increase after the Fontan operation. Cavity measures decreased most dramatically after the Fontan operation, with less dramatic and equivalent changes noted after the hemi-Fontan operation and ventricular septal defect closure. Posterior wall thickness to cavity diameter ratios were equivalent in all before operation, increased after operation, and were greatest after the Fontan operation. CONCLUSIONS: Changes in ventricular geometry identified as an increase in wall thickness and a decrease in cavity dimension are most dramatic after the Fontan operation. Changes seen after the hemi-Fontan operation are of a milder degree, which may in part explain the excellent clinical course after this operation.

Interrupted aortic arch. Impact of subaortic stenosis on management and outcome.

Interrupted aortic arch (IAA) is often related developmentally to subaortic obstruction (SAO). When severe, SAO must be addressed in surgical management of IAA. From 1990 to 1993, 25 neonates presented for initial surgical management of IAA complexes. Associated lesions were ventricular septal defect (VSD) with or without atrial septal defect (19 patients), truncus arteriosus (3 patients), tricuspid atresia with transposition of the great arteries (1 patient), aortic atresia with VSD (1 patient), and d-transposition of the great arteries with VSD (1 patient). Overall hospital mortality was 20% (five deaths). One death was related to sepsis and two to sudden hemodynamic decompensation (a 2-kg premature infant after arch repair and VSD closure and a neonate with IAA-truncus arteriosus after arch repair and truncus repair with aortic root replacement). Two deaths were related to low cardiac output in patients with severe subaortic narrowing (< 3 mm by two-dimensional echocardiography), which was not addressed surgically. Of 10 additional patients judged preoperatively to have severe SAO, 1 underwent resection of the infundibular septum together with VSD closure and arch reconstruction, and 9 underwent a modification of Norwood's operation with arch reconstruction and proximal pulmonary artery to aortic anastomosis (7 with systemic to pulmonary artery shunts and 2 with right ventricle to pulmonary artery outflow tract reconstruction). One patient died 2 months after surgery of staphylococcal sepsis. All 9 others were discharged well. Subaortic narrowing is a physiologically important element of IAA complexes. When SAO is severe, satisfactory initial palliation can be achieved by a modification of Norwood's operation.

Results of Norwood's operation for lesions other than hypoplastic left heart syndrome.

Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.

Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery.

OBJECTIVES: This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect-dependent systemic flow. BACKGROUND: Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined. METHODS: The preoperative and postoperative echocardiograms of 18 patients 14.9 +/- 22.8 months old (mean +/- SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 +/- 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured. RESULTS: Mean ventricular septal defect area indexed to body surface area diminished by 36 +/- 23% (3.1 +/- 2.7 to 2.0 +/- 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 +/- 19%, p < 0.01) than after pulmonary artery banding (25 +/- 28%, p = 0.22). CONCLUSIONS: In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding.

The pharmacokinetics of injectable allopurinol in newborns with the hypoplastic left heart syndrome.

OBJECTIVE: The purpose of this investigation was to determine the pharmacokinetic disposition of intravenous allopurinol and its metabolite oxypurinol in neonates with the hypoplastic left heart syndrome (HLHS) and to evaluate the subsequent degree of xanthine oxidase inhibition using serum uric acid as a marker. METHODS: Pharmacokinetic data were evaluated in 12 stable preoperative neonates with HLHS after a single intravenous allopurinol administration of 5 mg/kg or 10 mg/kg. Pharmacokinetic parameters were determined for elimination half-life, clearance, volume of distribution, and mean residence time. Xanthine oxidase inhibition, measured by serum uric acid reduction, was also measured. RESULTS: Pharmacokinetic parameters revealed no statistically significant differences between a 5-mg/kg and 10-mg/kg dose of intravenous allopurinol on elimination half-life, clearance, volume of distribution, and mean residence time. Mean serum uric acid levels were significantly reduced from baseline by 39.99 and 42.94%, respectively, in the 5- and 10-mg/kg treatment groups. DISCUSSION: The enzyme xanthine oxidase plays a key biochemical role in the generation of toxic oxygen-derived free radicals during ischemia-reperfusion conditions. Allopurinol and its active metabolite oxypurinol inhibit xanthine oxidase, and significantly reduce the conversion of hypoxanthine to xanthine and xanthine to uric acid. Cell injury may be caused by toxic oxygen free radicals produced by ischemia-reperfusion injury such as could occur during the repair of HLHS under hypothermic total circulatory arrest. We hypothesize that allopurinol may provide protection from cellular injury in this clinical context.

Early changes in ventricular geometry and ventricular septal defect size following Rastelli operation or intraventricular baffle repair for conotruncal anomaly. A cause for development of subaortic stenosis.

BACKGROUND: Obstruction to left ventricular outflow can be seen after surgical repair of congenital heart disease in which a ventricular septal defect (VSD) is closed by means of a baffle to the systemic great artery arising from the right ventricle (Rastelli operation, intraventricular repair, for conotruncal anomaly). We investigated the hypothesis that obligatory volumetric changes that occur after this operation lead to acute alterations in ventricular geometry and VSD size, resulting in subsequent subaortic stenosis in patients who were thought before operation to have a large, nonrestrictive VSD orifice. METHODS AND RESULTS: Preoperative and postoperative echocardiograms and medical records of 24 patients with conotruncal anomaly who underwent conventional Rastelli operation or intraventricular repair in which the VSD was used as part of a new left ventricular outflow were reviewed. Eleven patients had transposition of the great arteries with pulmonic stenosis, 7 had double-outlet right ventricle, and 6 had subaortic atresia or stenosis with a normal-size left ventricle and underwent Norwood's palliation in infancy. All had large, nonrestrictive VSDs at preoperative cardiac catheterization. The mean age at the time of surgery was 32 +/- 24 months. The following measurements were made from two-dimensional echocardiographic images obtained before and 5 +/- 4 days after surgery from the subcostal views at end diastole: (1) VSD diameter; (2) short-axis left ventricular internal diameter (LVID); (3) left ventricular posterior wall thickness (LVPW); and (4) systemic great artery diameter (arising from the right ventricle). VSD diameter diminished significantly after surgery (11.6 +/- 3.6 versus 10.1 +/- 3.7 mm, P < .0001), as did LVID (34.9 +/- 5.0 versus 31.7 +/- 5.1 mm, P < .001). LVPW thickness increased significantly (5.7 +/- 1.0 versus 6.7 +/- 1.1 mm, P < .0001), while great artery diameter was unchanged (16.2 +/- 4.0 versus 16.7 +/- 3.8 mm, P = NS). Percent change in VSD dimension correlated with percent change in LVPW/LVIDD ratio (degree of ventricular "contraction"). Nine patients subsequently developed subaortic obstruction at the VSD orifice level and had a greater degree of early diminution in VSD size (21 +/- 8% versus 10 +/- 8%, P < .002) as well as postoperative change in LVPW/LVID ratio (0.24 +/- 0.04 versus 0.20 +/- 0.02, P < .002) than those who did not develop subsequent subaortic obstruction. CONCLUSIONS: The left ventricle undergoes geometric change after Rastelli operation or intraventricular repair, surgeries in which the VSD is used as the new left ventricular outflow. These changes are manifested as increased wall thickness, decreased cavity dimensions, and a decrease in VSD size. Patients who subsequently develop left ventricular outflow obstruction have the greatest degree of ventricular contraction and VSD diminution early after surgery.

Experimental model of single ventricle. Influence of carbon dioxide on pulmonary vascular dynamics.

BACKGROUND: Important in the postoperative management of neonates with single-ventricle complexes, such as hypoplastic left heart syndrome and pulmonary atresia with intact ventricular septum, is the fact that the pulmonary circulation is connected to the systemic circulation via a shunt. The distribution of flow to the pulmonary and systemic vascular beds depends directly on the pulmonary-to-systemic vascular resistance ratio. Changes in this ratio result in alterations in flow that have an impact on survival. METHODS AND RESULTS: PICO2 has a potent effect on pulmonary vascular resistance. In this study, the addition of CO2 to inspired gas (PICO2) is assessed as a means of modulating the ratio of pulmonary-to-systemic vascular resistance and thus flow, while avoiding the consequences of hypoventilation. Thirteen neonatal piglets (age, 17 to 30 days) were subjected to atrial septectomy, patch closure of the tricuspid valve, and creation of a 4-mm systemic-to-pulmonary arterial shunt to mimic the physiology of single-ventricle complexes. Pulmonary and systemic flows; aortic, pulmonary artery, and atrial pressures; and arterial blood gases were measured with the addition of different levels of CO2 to the inspired gases (PICO2 = 0 to 35 mmHg). In all animals, pulmonary vascular resistance (PVR) increased in direct correlation with PICO2. In group 1 (n = 8), PVR increased in direct correlation with PICO2 and PACO2, and inversely with respect to pH (r = .98, .74, and .83, respectively). Group 2 animals (n = 5) received infusions of Tham to buffer hydrogen ion and compensate for respiratory acidosis. PVR again increased in direct correlation with FICO2 (r = .97). In both groups, systemic vascular resistance (SVR) was less directly influenced by FICO2 (r = .53). CONCLUSIONS: CO2 exerts a vasoactive influence on pulmonary vasculature that is largely independent of SVR and pH. Regulating PICO2 is an effective means of achieving a favorable ratio of pulmonary-to-systemic vascular resistance and thus flow in the fragile physiology encountered in neonates with single-ventricle complexes.