Use of autogenous aortic and main pulmonary artery flaps for repair of anomalous origin of the right pulmonary artery from the ascending aorta.
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Biomedical subjects
Publications and source records attributed to F L Hanley.
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BACKGROUND: For patients with complex left ventricular outflow tract obstruction, including hypoplastic aortic anulus with or without severe diffuse subaortic stenosis, various aortoventriculoplasty procedures (e.g., Konno procedure and its modifications; extended aortic allograft root replacement) are important management options. In younger patients, however, reoperation for valve replacement is inevitably required, and anticoagulation issues pose additional problems. The pulmonary autograft provides a promising option for aortic valve replacement as part of the aortoventriculoplasty procedure in children. Long-term follow up shows that the pulmonary autograft functions well as the systemic arterial (neoaortic) valve and that valve growth occurs. METHODS: Between July 1993 and May 1995, 11 patients 4 days to 17 years old (median 12 months) underwent aortoventriculoplasty with pulmonary autograft (Ross-Konno procedure). The diagnoses were aortic stenosis with or without subaortic stenosis (n = 8), Shone complex (n = 2), and interrupted aortic arch with subaortic stenosis (n = 1). On average, 1.9 previous interventions had been performed per patient, including a previous Konno procedure in one patient. The aortic root was replaced with a pulmonary autograft valve. The left ventricular outflow tract was enlarged with a Dacron polyester fabric patch in two patients, with an allograft aortic patch in two patients and a right ventricular infundibular free wall muscular extension harvested in continuity with the autograft in seven patients. RESULTS: Intraoperative transesophageal echocardiographic assessment revealed mild aortic insufficiency in one patient. One patient had a residual left ventricular outflow tract gradient of 15 mm Hg. Significant complications were cardiac tamponade from bleeding (n = 1) and complete heart block necessitating a permanent pacemaker (n = 1). Follow-up ranged from 2 weeks to 16 months. To date, there have been no late deaths or reoperations. Follow-up echocardiography revealed mild autograft insufficiency in one patient and a 16 mm Hg residual left ventricular outflow tract gradient in one patient. CONCLUSIONS: Initial experience suggests that aortoventriculoplasty with the pulmonary autograft is an excellent alternative for young patients with complex left ventricular outflow tract obstruction. Because the pulmonary autograft has been shown to grow after implantation, reoperation on the left ventricular outflow tract is likely to be avoided.
The cases of five patients with previous Senning (n = 4) or Mustard (n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m2, versus 55.6 gm/m2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount (n = 1) or disappeared completely (n = 3).
OBJECTIVES: A reproducible fetal animal model of single-ventricle physiology was created to examine the effects of pharmacologic agents commonly used in the perinatal and perioperative intensive care management of patients with a single ventricle. BACKGROUND: Single-ventricle physiology is characterized by parallel pulmonary and systemic circulations, with effective blood flow to each determined by the relative resistances in the pulmonary and systemic vascular beds. Perinatal and perioperative management of these patients is largely based on empiric observations and differs considerably between institutions and is further complicated by the transitional physiology of the newborn. The lack of animal models of single-ventricle physiology has hindered the understanding of this problem. METHODS: A 10-mm, Damus-Kaye-Stansel-type aortopulmonary anastomosis was created in 10 fetal sheep at 140 +/- 1.2 days of gestation. The main pulmonary artery was ligated distally, and pulmonary blood flow (Qp) was provided through a 5-mm aortopulmonary shunt. Eight lambs were delivered at term and placed on cardiopulmonary bypass (30 min) 48 to 72 h after birth. Pharmacologic interventions (0.1 microgram/kg body weight per min of epinephrine, 2 mEq/kg of sodium bicarbonate and 10 mg/kg of calcium chloride) were performed before and after bypass, and hemodynamic responses were observed. The response to the epinephrine bolus was determined only in the postbypass study. RESULTS: Both before and after bypass, epinephrine infusion and calcium and bicarbonate administration increased Qp and systemic blood flow (Qs) (total cardiac output) but produced only small changes in the Qp/Qs ratio (-0.5% to -7.3% change). With the epinephrine bolus, Qp increased enormously, and the Qp/Qs ratio increased by 584% (p < 0.001). CONCLUSIONS: In neonatal lambs with single-ventricle physiology created in utero, epinephrine infusion and calcium and bicarbonate administration increased total cardiac output without significantly compromising the Qp/Qs ratio. However, epinephrine bolus seems to be hemodynamically detrimental in circumstances of single-ventricle physiology and should be used with caution and probably in relatively lower doses in the resuscitation of patients with single-ventricle physiology. Further investigation of the dose-dependent effects and the effects of prolonged administration of common pharmacologic agents will enable better management of patients with single-ventricle physiology.
BACKGROUND: Progressive metabolic acidosis, hypoxia, and hypercarbia develop rapidly after fetal cardiac bypass mainly as a result of an increase in placental vascular resistance and a decrease in placental blood flow. A number of factors including fetal stress, priming substances, and extracorporeal surfaces have been identified as possible stimuli causing this placental dysfunction. The purpose of this study was to examine the effects of avoiding priming volume and minimizing extracorporeal surface area on placental hemodynamics and function. METHODS: Fetal sheep (n = 16) at 118 to 122 days of gestation were subjected to cardiac bypass for 30 minutes using either an in-line axial-flow pump (Hemopump group: n = 8, no prime) or a roller pump with a venous reservoir (control group: n = 8, priming volume = 150 mL). After bypass, the fetuses were observed for 90 minutes. Placental blood flow and combined ventricular output were continuously measured with ultrasonic flow probes, and fetal blood gases were measured at specific intervals. RESULTS: Three fetuses in the control group died during the study, whereas all 8 fetuses in the Hemopump group remained in stable condition throughout the study period. During and after bypass, placental blood flow was significantly higher (p < 0.0001) and placental vascular resistance was significantly lower (p < 0.0001) in the Hemopump group than in the control group. Arterial pH and partial pressure of arterial oxygen declined significantly less (p < 0.0001), and partial pressure of arterial carbon dioxide increased significantly less (p = 0.0002) in the Hemopump group than in the control group. CONCLUSIONS: Reducing the extracorporeal surface area and avoiding external priming substances preserves placental hemodynamics after fetal cardiac bypass. An in-line axial-flow pump is useful in miniaturizing the bypass circuits for potential use in fetal cardiac surgery.
The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.
Although it has been almost 30 years since Ross described his technique for aortic valve replacement using the pulmonary autograft, only recently has there been widespread interest in the use of the Ross procedure for aortic valve replacement in children with congenital heart disease. Since July 1992 we have performed the Ross procedure in 46 patients, 40 of whom were < or = 18 years of age at the time of surgery. In 10 children the procedure was combined with Konno ventriculoplasty, and in 3 others a Ross-Konno procedure was used for biventricular repair of borderline hypoplastic left heart syndrome. The only early deaths were in two of the patients with borderline hypoplastic left heart syndrome. There has been no late mortality, and two patients have required reoperation: one for recurrent distal aortic arch obstruction, and one for moderate-severe autograft insufficiency. Freedom from reoperation at 30 months is 89%. Aside from one patient with moderate autograft insufficiency and eight with mild regurgitation, all patients have trace or no aortic insufficiency at follow-up ranging from 1 to 38 months (median 22 months). The Ross procedure is an important advance in the treatment of aortic valve disease in pediatric patients. It is the only available replacement for the aortic valve that has growth potential, and there is ample evidence that the pulmonary autograft holds up well in the systemic circulation.
BACKGROUND: The neurological morbidity associated with prolonged periods of circulatory arrest has led some cardiac surgical teams to promote continuous low-flow cardiopulmonary bypass as an alternative strategy. The nonneurological postoperative effects of both techniques have been previously studied only in a limited fashion. METHODS AND RESULTS: We compared the hemodynamic profile (cardiac index and systemic and pulmonary vascular resistances), intraoperative and postoperative fluid balance, and perioperative course after deep hypothermia and support consisting predominantly of total circulatory arrest or low-flow cardiopulmonary bypass in a randomized, single-center trial. Eligibility criteria included a diagnosis of transposition of the great arteries and a planned arterial switch operation before the age of 3 months. Of the 171 patients, 129 (66 assigned to circulatory arrest and 63 to low-flow bypass) had an intact ventricular septum and 42 (21 assigned to circulatory arrest and 21 to low-flow bypass) had an associated ventricular septal defect. There were 3 (1.8%) hospital deaths. Patients assigned to low-flow bypass had significantly greater weight gain and positive fluid balance compared with patients assigned to circulatory arrest. Despite the increased weight gain in the infants assigned to low-flow bypass, the duration of mechanical ventilation, stay in the intensive care unit, and hospital stay were similar in both groups. Hemodynamic measurements were made in 122 patients. During the first postoperative night, the cardiac index decreased (32.1 +/- 15.4%, mean +/- SD), while pulmonary and systemic vascular resistance increased. The measured cardiac index was < 2.0 L.min-1.m-2 in 23.8% of the patients, with the lowest measurement typically occurring 9 to 12 hours after surgery. Perfusion strategy assignment was not associated with postoperative hemodynamics or other nonneurological postoperative events. CONCLUSIONS: After heart surgery in neonates and infants, both low-flow bypass and circulatory arrest perfusion strategies have comparable effects on the nonneurological postoperative course and hemodynamic profile.
BACKGROUND: The development of pulmonary hypertension and its associated increased vascular reactivity is a common accompaniment of congenital heart disease with increased pulmonary blood flow. Although the morphology of the pulmonary vascular changes is well described, the mechanisms of vascular remodeling and increased reactivity remain incompletely understood. METHODS AND RESULTS: To elucidate these mechanisms, we established an accurate and reliable experimental model of pulmonary hypertension with increased pulmonary blood flow. An aortopulmonary shunt was created with an 8.0-mm expanded polytetrafluoroethylene vascular graft in 11 late-gestation fetal lambs. At 1 month of age, shunted lambs had a pulmonary-to-systemic blood flow ratio of 2.2 +/- 1.2. Compared with 11 age-matched control lambs, mean pulmonary arterial pressure (44.8 +/- 11.7 versus 16.2 +/- 2.9 mm Hg) and the ratio of pulmonary to systemic arterial pressure were significantly increased (P < .05). Pulmonary vascular resistance was not significantly increased. The pulmonary vasoconstricting response to the infusion of U46619 (a thromboxane A2 mimic) or acute alveolar hypoxia also was augmented in the shunted lambs. Morphometric analysis of the barium-filled pulmonary artery bed revealed medial hypertrophy, abnormal extension of muscle distally into the walls of the intra-acinar arteries, and increased numbers of barium-filled intra-acinar arteries. CONCLUSIONS: In utero placement of aortopulmonary shunts reproduces the aberrant hemodynamic state of children with cogenital heart disease with left-to-right shunts; postnatal pulmonary hypertension, increased pulmonary blood flow, and vascular remodeling. In addition, the lambs have a unique paradoxical increase in pulmonary vascular volume that attenuates an increase in pulmonary vascular resistance. This experimental preparation provides a useful and consistent model for the study of the pathogenesis of pulmonary hypertension.
The performance of a primary bidirectional superior cavopulmonary shunt procedure in early infancy is attractive because it minimizes the number of operations needed before a Fontan procedure, avoids ventricular volume overload and its sequelae, and eliminates pulmonary artery distortion. However, concerns over elevated or labile pulmonary vascular resistance have limited its use in the first few months of life. Nine patients aged 1 to 4 months (5 patients, < 2 months) have undergone a primary bidirectional superior cavopulmonary shunt procedure between October 1992 and March 1994. Primary diagnoses were tricuspid atresia (n = 4), asplenia syndrome (n = 2), polysplenia syndrome (n = 1), double-outlet right ventricle (n = 1), and double-inlet left ventricle (n = 1). Associated lesions of immediate surgical importance were total anomalous pulmonary veins (n = 2), a restrictive atrial septum (n = 4), bilateral superior venae cavae (n = 5), and patent ductus arteriosus (n = 5). The surgical procedure consisted of unilateral (n = 4) or bilateral (n = 5) bidirectional superior cavopulmonary shunt and the repair of associated lesions. Of significance, in 4 of our first 5 patients a very limited additional source of pulmonary blood flow was provided because of a low arterial oxygen tension immediately after cardiopulmonary bypass. Pleural effusions developed in 2 of these 4 patients. In subsequent patients cardiopulmonary bypass was not used whenever possible or, if it was needed, use of an extra source of pulmonary blood flow was avoided.(ABSTRACT TRUNCATED AT 250 WORDS)
An infant with pulmonary atresia and intact ventricular septum is presented who, after initial patch reconstruction of the right ventricular outflow tract and bidirectional cavopulmonary anastomosis through a fifth median sternotomy, underwent an echocardiographically guided closed atrial septotomy, which resulted in marked long-term clinical improvement. The technique of intraoperative transesophageal echocardiography as used in the presented case represents an expanded role for this diagnostic modality in congenital cardiac surgery.
A modified technique of aortic root replacement with the pulmonary autograft is described with the purpose of eliminating native pulmonary artery tissue as a support structure for the autograft valve leaflets. Large coronary buttons are preserved to replace the native pulmonary sinuses completely, and the noncoronary aortic sinus is left in situ to externally buttress the noncoronary sinus of the pulmonary autograft.
BACKGROUND: The potential for growth and the proven long-term durability of the native pulmonary valve make it ideal for replacement of the diseased aortic valve, especially in growing children. The use of the autologous pulmonary valve can be further extended to patients with complex left ventricular outflow tract obstruction and to neonates and infants. METHODS: Between June 1993 and May 1995, 35 patients underwent the Ross procedure at our center. Of these, 15 (43%) had complex left ventricular outflow tract obstruction and 7 (20%) were infants, including 3 neonates. The autologous pulmonary valve was implanted as a root replacement with coronary reimplantation in all patients. Additional left ventricular outflow tract procedures performed were ventricular myectomy in 7 patients and a Konno type aortoventriculoplasty in 11 patients. RESULTS: There was one early death in a patient with borderline hypoplastic left heart syndrome. At a median follow-up of 9 months (range, 0.2 to 22 months) there were no late deaths or reinterventions. The autologous pulmonary valve function was excellent, with 1 (2.8%) patient having moderate insufficiency. CONCLUSIONS: Autologous pulmonary valve is an excellent option for aortic valve replacement in all age groups. Its use can be readily extended to neonates, infants, and patients with complex left ventricular obstruction requiring additional left ventricular outflow tract procedures.
BACKGROUND: Although primary repair of tetralogy of Fallot is increasingly undertaken in infancy, complete repair is generally performed in only selected symptomatic neonates. METHODS: From July 1992 through March 1995, 30 consecutive neonates and young infants with tetralogy of Fallot underwent routine primary repair. Group I (n = 10) consisted of patients with tetralogy of Fallot and pulmonary atresia (n = 5) or severe pulmonary stenosis (n = 5) who were duct dependent and were repaired in the neonatal period. Group II (n = 11) consisted of patients who were asymptomatic with arterial oxygen saturation between 75% and 90% (adequate pulmonary blood flow). Group III (n = 9) consisted of patients with "pink" tetralogy of Fallot (arterial oxygen saturation > 90%). Patients in groups II and III were electively scheduled for repair at about 2 months of age. RESULTS: The postrepair peak systolic right ventricular-to-peak systolic left ventricular pressure ratio did not correlate (p = 0.96) with the branch pulmonary artery size. One patient died 2 months after operation, despite good hemodynamics, of uncontrollable diffuse subcutaneous edema due to familial distichiasis lymphedema syndrome. There were no late deaths. At a median follow-up of 19 months, 1 patient underwent balloon dilation of branch pulmonary arteries. Follow-up echocardiography revealed a 30 to 60 mm Hg right ventricle-to-pulmonary artery gradient in 3 patients. CONCLUSIONS: Excellent early and midterm results can be accomplished with routine primary repair of tetralogy of Fallot in early infancy regardless of age, symptomatic status, coronary anatomy, and the size of branch pulmonary arteries as long as they arborize normally.
BACKGROUND: Sustained tachyarrhythmia resulting in fetal hydrops is often refractory to medical therapy. Fetal atrioventricular node ablation associated with epicardial fetal pacing has the potential to be an effective procedure for this morbid association. METHODS: To assess the feasibility of therapeutic fetal heart block, we developed a technique of intrauterine cryosurgical ablation of fetal atrioventricular node without the need for cardiac bypass in 8 fetal lambs. Complete heart block was obtained by applying the cryoprobe over the coronary sinus. Fetal pacing was then performed to allow fetal survival. RESULTS: Complete heart block was achieved in 100% of the fetal lambs. Postoperative evaluation revealed persistent atrioventricular block. The hearts were studied at different postoperative times. Morphologic evaluation of the area containing the cryosurgical lesion revealed varied extension of necrosis of the atrioventricular node and hemorrhage, with involvement of the His bundle and proximal right bundle branch. CONCLUSIONS: This procedure is technically feasible and offers an alternative approach to the treatment of drug-resistant, life-threatening fetal supraventricular tachyarrhythmias associated with hydrops fetalis.
Modified techniques of aortopulmonary anastomosis were performed in six neonates with atrioventricular and ventriculoarterial discordance [S,L,L], double-inlet left ventricle, and restrictive bulboventricular foramen area (mean index 1.10 cm2/m2) with unobstructed aortic arch (n = 3) or with hypoplasia (n = 2) or interruption (n = 1) of the aortic arch. In cases of unobstructed aortic arch, a flap of autogenous aortic tissue was used to augment the posterior aspect of the anastomosis of the main pulmonary artery to the ascending aorta, thus creating the potential for anastomotic growth; this technique is applicable regardless of the position of the ascending aorta relative to the main pulmonary artery. In case of levo-transposition of the aorta with hypoplasia or interruption of the aortic arch, a modified Norwood procedure was performed, in that the proximal ascending aorta was divided at the same level as the main pulmonary artery with subsequent homograft patch augmentation from the main pulmonary artery-ascending aorta anastomosis to the level of the proximal descending aorta; this technique avoids a spiraling incision of the aorta and therefore reduces the risk of torsion of the aortic root with its inherent risks of obstruction of the coronary circulation and aortic or pulmonary valve regurgitation. There was no early or late mortality. At a mean follow-up of 16 months, in all patients, there was unobstructed aortic outflow, as evidenced by echocardiographic absence of a significant ventricular-aortic systolic gradient (mean 4.5 +/- 4 mm Hg) and absence of distal aortic arch obstruction. There was no evidence of aortic or pulmonary valve regurgitation. The reported modified techniques provide effective relief of restrictive bulboventricular foramen and aortic obstruction in [S,L,L] hearts.
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Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The ventricular septal defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right ventricular/left ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open ventricular septal defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting ventricular septal defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required.(ABSTRACT TRUNCATED AT 400 WORDS)