Waterston Shunt Research Articles

Unravelling the Convoluted Story of Perioperative Care in Three-year-old Child with Tetralogy of Fallot Undergoing Repair Surgery

Tetralogy Of Fallot (TOF), one of the most prevalent cyanotic congenital heart diseases in children. Single step corrective surgery, early on in life, provides a fair expectation of favourable outcome in these individuals. However, complex, and skilled anaesthetic management is required by experienced team of healthcare providers. Preoperative surgical preparation, intraoperative key anaesthesia principles and postoperative care intensive care unit are all perioperative considerations in these patients. This article reports challenges faced in the perioperative anaesthesic management of a 3-year-old child having uncorrected TOF, who underwent Waterston shunt and later Intracardiac Repair (ICR). He presented with postoperative complication like cyanotic spell, gastrointenstinal bleeding, sepsis; but later recovered successfully.

Experience with Palliative Procedures for Congenital Cyanotic Heart Diseases in Ghana: A 20-Year Review

Background:Currently, in the management of congenital cyanotic heart diseases with favourable anatomy, primary correction is the preferred choice. But palliative procedures have still maintained an important role in resource-poor regions of the world.We sought to analyze our institutional results for such palliation over a 20-year period. The purpose of the study was to provide information that would guide both practitioners and referring doctors on the future management of congenital cyanotic heart diseases, and also to provide data for comparative studies in the sub region in the future.Patients and Methods: A retrospective study was carried out in the National Cardiothoracic Centre involving all patients who had palliative procedures for congenital cyanotic heart diseases from January 1992 to December 2011.Results: There were 264 patients, 59% males and 41% females. The case load increased from 2 cases per year in the first year to 34 in the last year of the study. The age range was 4 months – 42 years, with a mean of 7.1 ± 6.9 years. Tetralogy of Fallot comprised 257 (97.4%) of the cases, tricuspid atresia 4 (1.5%), and double outlet right ventricle 3 (1.1%). The modified Blalock-Taussig shunt (MBTS) was performed in 262 (92.2%), and the Waterston shunt in 2 (0.8%) of the cases. The overall complication rate was 11.8%, comprising shunt occlusion 7.6%, bleeding requiring re-exploration 0.8% and early mortality of 3.4%.Conclusion: The modified Blalock-Taussig shunt provided good palliation for congenital cyanotic heart diseases in this environment. We consider it a suitable management alternative when financial and logistic constraints delay primary repair in resource-poor settings.

Inside-Out Venous Access–Assisted Transcatheter Pulmonary Valve

A 39-year-old man with congenital heart disease presented with right heart failure due to severe pulmonic regurgitation. The patient was born with pulmonary atresia and intact ventricular septum. He was managed with atrial septostomy and a Waterston shunt, followed by patch augmentation of his

Combined strategy of Waterston shunt percutaneous occlusion and medical treatment with sildenafil for management of pulmonary hypertension in an adult patient with corrected tetralogy of Fallot.

Combined strategy of Waterston shunt percutaneous occlusion and medical treatment with sildenafil for management of pulmonary hypertension in an adult patient with corrected tetralogy of Fallot.

Congenital Heart Defects: Early Surgical Correction and Heart Failure? Brief History

The first successful cardiac surgical procedure was performed by Robert Gross in 1938 in Boston. A 7 year-old patient underwent ligation of the ductus arteriosus [1]. In 1945, Crawford carried out the first correction for coarctation of the aorta in Sweden [2,3].Technological development that allowed for the introduction of extracorporeal circulation devices led to the beginning of open heart surgery. The first heart surgery using an extracorporeal circulation device was performed by Kirklin. Extracorporeal circulation devices had multiple negative effects on the physiology of the infant and the newborn, so that at the beginning of pediatric heart surgery, the delay of surgical correction was preferred. Initially, these devices required an extremely high priming volume that exposed the child to exanguinotransfusion. Inflammation mediators [4] caused excessive vascular permeability and tissue edema, all the more so as the age of the child was younger at the time of surgery.At first, anesthesia and intensive care for children were practically non-existent, and diagnosis was made using invasive techniques (cardiac catheterization), which most frequently generated complications. Surgical kits did not contain microsurgical instruments required for working on the fragile heart tissue of the newborn and infant. Thus, a number of palliative procedures were developed, in order to help the patient survive until the performance of surgical correction.The pathophysiology of congenital heart defects includes 3 main categories. There may be a volume load in which one or both ventricles must pump more blood than normal. This usually happens because of an increased pulmonary blood flow, as a result of a septal defect. There may be a pressure load of one or both ventricles. This can be the result of an obstruction at the outlet of the ventricle. The third possibility is cyanosis that can be secondary to a reduction in pulmonary blood flow or inadequate mixing between the two parallel circulations.Systemic pulmonary shunt reduces cyanosis by increasing pulmonary blood flow. Although from a conceptual point of view it is an easy procedure, systemic pulmonary shunt can be a challenge for the surgeon. Assessing the size of the shunt depending on the weight of the child is most important. The size of the shunt must take into consideration the subsequent development of the child, but it must not be too large in order to avoid postoperative ventricular volume overload. The Blalock-Taussig shunt was introduced by the surgeon Alfred Blalock and the cardiologist Helen Taussig [5]. The original BT shunt directly connected the sectioned subclavian artery to the right pulmonary artery and was of an adequate size to substitute for the pulmonary blood flow deficiency. In addition, this shunt had growth potential and thus, it could pathophysiologically support the patient for many years. The Waterston shunt is an anastomosis between the ascending aorta and the right pulmonary artery [6]. This shunt has the disadvantage to produce excessive pulmonary blood flow and a distortion of pulmonary arteries.The Potts shunt consists of the anastomosis of the descending aorta and the left pulmonary artery. This shunt has all the disadvantages of the Waterston shunt and in addition, it is extremely difficult to remove at the time of surgical correction.The modified BT shunt, which consists of the anastomosis of the arterial brachiocephalic trunk with the right pulmonary artery, by means of a vascular prosthesis, was introduced by deLeval [7]. This shunt was initially performed in the left subclavian artery and the left pulmonary artery through thoracotomy. It has the following advantages: it does not cause pulmonary artery distortion and pulmonary overload.High blood pressure and flow in pulmonary circulation, most frequently due to a septal defect, may lead in time to pulmonary hypertension and pulmonary vascular disease. In 1950, Muller and Dammann introduced pulmonary artery banding in order to reduce high blood pressure and flow in pulmonary circulation [8]. Like in the case of shunts, banding does not allow for an increase in diameter, so that in time, with the child’s growth, this becomes increasingly tight. It can also result in pulmonary artery distortion, so that correction and the removal of banding should be initiated as soon as possible after the regression of pulmonary hypertension.In 1950, Blalock and Hanlon introduced surgical atrial septectomy for children with transposition of the great vessels with insufficient mixing [9]. Nowadays, this septectomy is an interventional procedure performed through the rupture of the septum with a balloon catheter, according to the procedure described by Rashkind [10,11].

Intervening for RPA stenosis following Waterston shunt: Importance of anatomical definition of the coronary arteries

Right pulmonary artery (RPA) stenosis following Waterston shunt is common. We report a case of RPA stenosis many years following tetralogy of Fallot repair with take-down of a Waterston shunt and demonstrate an unusual and important anatomic variant of proximity of the left coronary artery to the mid-portion of the RPA.

Use of panoramic body surface electrocardiographic for mapping of tachyarrhythmias in adult patients with congenital heart disease

Introduction: In patients with congenital heart disease the complex underlying anatomy and dilatation of the cardiac chambers demands extensive invasive intracardiac mapping. We assessed the clinical utility of a novel noninvasive, single beat simultaneous electrocardiographic mapping system (ECVUE™) in diagnosing complex and/or multiple tachycardias in this population. Methods: Consecutive patients with congenital heart disease and previous endocardial ablation procedures were selected. Noninvasive body surface ECVUE™ simultaneous spatial mapping was performed both pre- and intra-procedurally to localize the arrhythmia origin and to guide ablation. Results: Six patients (4 female) with a median age of 28 years were studied. Patients' anatomical background included left atrial isomerism, DTGA post arterial switch, pulmonary atresia, surgically repaired partial AVSD, pulmonary stenosis with Waterston shunt and Ebstein's anomaly. Non-invasive ECVUE™ evaluated 13 arrhythmias across the 6 patients (2.17 arrhythmias/patient) and correctly diagnosed the region of interest in 12 of 13 (92%) arrhythmias. Median procedure duration and fluoroscopy time were 233 min and 14 min, respectively. Remote magnetic navigation was used in 2 procedures. Acute ablation success was achieved in 5 of 6 patients, and in 11 of 13 arrhythmias. ![Figure][1] Figure 1 Conclusions: Non-invasive panoramic mapping, via ECVUE™, is a novel tool for evaluating complex arrhythmias, particularly helpful in patients with congenital anomalies and previously failed ablation. It can be used in conjunction with other technologies such as remote magnetic navigation, facilitates ablation of multiple arrhythmias in single procedure, with no longer than expected procedure length or fluoroscopy time. [1]: pending:yes

Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures. We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries. Methods From 2001 to 2012, a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures. From among them, 31 patients had pulmonary atresia with ventricular septal defect (VSD) and the other 10 cases had complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, a Glenn procedure was performed. A modified Blalock-Taussig shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic. If the pulmonary arteries were severely hypoplastic, a Melbourne shunt was performed. Systemic pulmonary artery shunts were performed bilaterally in 25 cases. A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases. Major aortopulmonary collateral arteries were unifocalized in six cases, ligated in two cases and interventionally embolized in two cases. There was one early death because of cardiac arrest and the hospital mortality was 2.4%. Results Five patients suffered from postoperative low cardiac output syndrome, three had perfusion of the lungs, and two pulmonary infections. Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits. The mean follow-up time was 25 months. The pre- and the post-operation left pulmonary indices were (8.13±3.68) vs. (14.9±6.21) mm2/m2. The pre- and post-operation right pulmonary indices were (12.7±8.13) vs. (17.7±7.78) mm2/m2. The pre- and post-operational pulmonary indices were (20.87±9.43) vs. (32.6±11.7) mm2/m2. They were all significantly increased (P <0.001). The diameter of the pulmonary artery increased after the modified Blalock-Taussig shunt ((5.51±0.94) mm2/m2 pre-operation vs. (7.01±1.97) mm2/m2 post-operation), the modified Waterston shunt ((5.70±3.96) mm2/m2 pre-operation vs. (9.17±3.62) mm2/m2 post-operation) and the Melbourne shunt ((2.17±0.41) mm2/m2 pre-operation vs. (7.35±2.49) mm2/m2 post-operation) (all P <0.05). Bilateral pulmonary arteries developed well as compared to their pre-operation development. Hemoglobin decreased from (194±27) to (174±24) g/L (P <0.05) and peripheral oxygen saturation increased from (65±11)% to (84±6)% (P <0.001). During the follow-up of 27 to 49 months, ultimate complete repair was performed in four cases and one patient underwent a Glenn procedure. Conclusions The procedures should be considered on a case to case basis in patients having hypoplasia of the pulmonary arteries with cyanotic congenital heart defects. Combined palliative operations could be an adequate strategic treatment.

Editorial Comment: Natural and modified history of single-ventricle physiology in adult patients

The benefits and long-term effects associated with single-ventricle (SV)/Fontan palliation are increasingly appreciated as the first generation of recipients with SV physiology enters their fourth decade of follow-up [1]. The suboptimal preload with systemic venous hypertension, abnormal cardiac contractility and elevated afterload have all contributed to the relentless but gradual deterioration in palliated patients with SV hearts. In the grown-up population, it is increasingly important to track late complications and deaths due to arrhythmias, thromboemboli, protein-losing enteropathy (PLE), worsening cyanosis and heart failure, and to elucidate the risk factors for them such as right ventricle morphology, higher Fontan pressure and era effects. In this issue, Angeli et al. [2] reported their single-centre experience with 62 patients aged older than 16-years with SV physiology. It would have been beneficial if the article had provided robust data about the natural history of SV (separated from the effect of surgical treatment), the detailed management strategy of obsolete operations such as atriopulmonary connection and any progress in the non-contemporary management. Only three patients were in the natural history group. Three patients were in the palliated group (one Blalock Taussig shunt, one Waterston shunt and one pulmonary artery (PA) band). Fifty-six patients were in the modified history group [two bidirectional cavopulmonary anastomosis (BCPA), eight classical Fontan procedure and 46 total cavopulmonary anastomosis (TCPC)]. During the follow-up (mean 8 ± 9.1 years), three patients died (two with PLE post-TCPC and one with failed BCPA). Five patients (following PA banding, BCPA and atriopulmonary connection (APC) each in one patient, respectively, and TCPC in two patients) with a combination of worsening heart failure, significant atrioventricular valvular regurgitation, tachyarrhythmias and PLE, underwent heart transplantation with no early mortality. Seven patients underwent TCPC conversion for worsening NYHA Class III–IV, with atrial fibrillation (AF) in five patients and conduit thrombosis in one patient (mean age at conversion: 22 years) with no early mortality. There was reported improvement following conversion, however, three patients remained in AF and two patients with PLE. Thirty-three patients had magnetic resonance imaging for a functional and anatomical assessment, and 54 patients underwent CPET. The quality-of-life assessments (SF-36 and CHJD-TAAQOL) using telephone interviews were performed in 51 patients. The actuarial freedom from death and transplantation was 95% at 20 years of age, 75% at 30 years of age and 50% at 35 years of age. To provide the data relevant to the adults with Fontan palliation, the age selection in this paper was limited to those aged older than 16 years at the time of sampling. Considering that many such patients had surgery in an earlier era (1975–1985), it is perhaps surprising that there was no perioperative mortality. I can only make the assumption that the patients’ selection was probably based on a group of patients who survived the surgical procedure with ‘very good’ Fontan haemodynamics and had freedom from death or transplantation in the first 16 years of life. I personally believe that it would be more meaningful if the starting point was the age at the time of first intervention or the number of years since the Fontan procedure. Encouragingly, there were no early surgical mortality rates for either transplantation or TCPC conversion. However, clinical experience has shown that the role of the TCPC conversion is limited because of the dwindling population of grown-up patients with haemodynamics suitable for these physiological/surgical innovations. Heart transplantation as an exit strategy remains intrinsically limited by immunologically-related issues and extrinsically limited by the lack of donor availability and surgical complexity. Other options, such as solid organ xenotransplantation, have been on the horizon for many years and sadly will remain there for many more years to come. The novel approach of mechanical-assist devices [3] to offer an appropriate energy source to overcome the paradox Fontan physiology because of systemic venous hypertension imposed with concomitant pulmonary arterial hypotension would require formal clinical trials. Apart from the well-recognized sequalae of Fontan circulation, it is important to appreciate that normal ageing, pregnancy, acquired heart diseases and others would also have an impact on their quality of life.

Successful Hybrid Rescue of Occluded Pulmonary Artery in Pulmonary Atresia

Hybrid cardiac procedures are a result of synergistic cooperation between the cardiologist and surgeon. They are a combination of surgical repair and intraoperative catheter-based interventions, and may provide an effective option for the repair of complex congenital heart lesions. We present a 30-year-old man with pulmonary atresia and a ventricular septal defect. He previously had a Waterston shunt placed at 10 days of age and a right pulmonary artery shunt at 4 years of age. He had ventricular septal defect closure with a 23-mm homograft conduit connecting the right ventricular outflow tract to the main pulmonary artery confluence at 9 years of age. The patient presented to us 21 years later with pulmonary conduit obstruction, right pulmonary artery stenosis, and an occluded left pulmonary artery (LPA; Figures 1, 2A, and 2B). The catheterization demonstrated right ventricular pressure (70% of systemic arterial pressure) at 89/14 mm Hg. There was a 28-mm Hg gradient to the right pulmonary artery, and the LPA was occluded. A 7-mm distal LPA was demonstrated by left pulmonary vein wedge angiogram. Given the long occlusion between the conduit and the lower LPA, we were concerned that the LPA would not be accessible for direct patch augmentation, and hence decided to perform a hybrid procedure; …

Przeznaczyniowe zamknięcie czynnego zespolenia Waterstona u chorego po korekcji wrodzonej wady serca –

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Demkow M, Biernacka E, Kwaśniak D, et al. Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report. Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej. 2011;7(3):261-264. doi:10.5114/pwki.2011.24746. APA Demkow, M., Biernacka, E., Kwaśniak, D., Zaborowska, E., Petryka, J., & Stępińska, J. et al. (2011). Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report. Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej, 7(3), 261-264. https://doi.org/10.5114/pwki.2011.24746 Chicago Demkow, Marcin, Elżbieta Katarzyna Biernacka, Dominika Kwaśniak, Emilia Zaborowska, Joanna Petryka, Janina Stępińska, and Piotr Hoffman. 2011. "Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report". Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej 7 (3): 261-264. doi:10.5114/pwki.2011.24746. Harvard Demkow, M., Biernacka, E., Kwaśniak, D., Zaborowska, E., Petryka, J., Stępińska, J., and Hoffman, P. (2011). Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report. Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej, 7(3), pp.261-264. https://doi.org/10.5114/pwki.2011.24746 MLA Demkow, Marcin et al. "Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report." Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej, vol. 7, no. 3, 2011, pp. 261-264. doi:10.5114/pwki.2011.24746. Vancouver Demkow M, Biernacka E, Kwaśniak D, Zaborowska E, Petryka J, Stępińska J et al. Case reportPercutaneous closure of Waterston shunt in an adult after correction of congenital heart disease – a case report. Advances in Interventional Cardiology/Postępy w Kardiologii Interwencyjnej. 2011;7(3):261-264. doi:10.5114/pwki.2011.24746.

Combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

To explore the clinical experiences of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries. From August 2001 to September 2009, 31 patients with cyanotic congenital heart defects and intractable hypoplasia of pulmonary arteries underwent surgical procedures. Among them, 26 patients were pulmonary atresia with ventricular septal defect and the other 5 cases complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, Glenn procedure was performed. Modified Blalock-Taussig or Waterston shunt was performed if pulmonary arteries had hypoplasia. If the pulmonary arteries were of severe hypoplasia, Melbourne shunt was performed. Systemic-pulmonary artery shunts were performed bilaterally in 23 cases. Systemic-pulmonary shunt was performed in one side and Glenn procedure contralaterally in 8 cases. There was one early death because of cardiac arrest. The number of patients suffered from low cardiac output syndrome, perfusion lung and pulmonary infection postoperatively was 5, 3 and 2, respectively. Systemic-pulmonary shunts were reperformed after the original operation in 3 cases because of occlusion of conduits. The mean follow-up time was 25 ± 16 months (6 - 72 months). Left pulmonary index (8.1 ± 3.7 vs 14.9 ± 6.2), right pulmonary index (12.7 ± 8.1 vs 17.7 ± 7.8) and pulmonary index (20.9 ± 9.4 vs 32.6 ± 11.7) increased significantly (all P < 0.001). The pulmonary diameter increased significantly after modified Blalock-Taussig shunt (5.5 ± 1.0 vs 7.0 ± 2.0), modified Waterston shunt (5.7 ± 4.0 vs 9.2 ± 3.6) and melbourne shunt (2.2 ± 0.4 vs 7.4 ± 2.5) (all P < 0.05). Bilateral pulmonary arteries developed well compared with that of preoperative condition. Hemoglobin decreased from (194 ± 27) g/L to (174 ± 24) g/L (P < 0.05) and peripheral oxygen saturation increased from (65 ± 11)% to (84 ± 6)% (P < 0.001). During the follow-up ultimate complete repair were performed in 3 cases and one patients underwent Glenn procedure. The procedures should be considered for hypoplasia of pulmonary arteries in cyanotic congenital heart defects. Combined palliative operation is an adequate therapy.

Real-Time 3-Dimensional Echocardiography for Follow-up of Waterston Shunt

A 37-year-old man presented with Eisenmenger's reaction. He had progressive cyanosis and signs of right-sided heart failure. In early childhood he was palliated with a Waterston shunt for cyanotic congenital heart disease. Two-dimensional echocardiography provided only a rudimentary view of his heart's anatomy and hemodynamics. Real-time three-dimensional echocardiography (RT3DE) was superior in visualizing both the size and the shape of the large shunt. RT3DE easily identifies and quantifies a key pathogenic factor for pulmonary hypertension with irreversible vascular damage in such patients. If applied early, RT3DE may prove to be a valuable follow-up tool.

Echocardiogram‐gated multislice spiral CT in functionally univentricular heart following long‐lasting Waterston–Cooley anastomosis

Waterston-Cooley anastomosis may be carried out in patients with tricuspid atresia to provide pulmonary perfusion. It is associated with several complications, including preferential blood flow to the right lung, hypoplasia of the left pulmonary artery, obstruction of the anatomosis or rupture of pulmonary aneurysms. We study a patient with thrombosis in the pulmonary arteries following surgical construction of a Waterston shunt in childhood. Imaging findings and clinical symptoms are discussed with emphasis on echocardiogram-gated multislice spiral CT.

Abstracts for the British Congenital Cardiac Association Annual Meeting: The Barbican, London, 24–25 November 2005: Poster Presentations: Assessment of the need for elective balloon atrial septostomy in patients with tricuspid atresia. a 25 year experience at the Yorkshire Heart Centre

Introduction: Significant variation exists in the perceived indications for balloon atrial septostomy in children with tricuspid atresia (TA). Many units advocate early, elective balloon atrial septostomy due to potential progressive narrowing of the interatrial opening, especially after the insertion of an arterial shunt. Our practice is to perform an atrial septostomy only in children with clinical and/or echocardiographic features of a restrictive interatrial communication. The aim of the study was to assess our practice by retrospective evaluation of patients born with tricuspid atresia with particular reference to the clinical fate of the atrial septum. More specifically the frequency of emergency atrial septostomy, the complication rate, the need for delayed septostomy or surgical resection and the longer term outcome were evaluated. Materials and methods: The study was a retrospective review (1980–2005) of the data on 49 live-born patients in our departmental database with a diagnosis of TA. One patient was excluded from the study due to referral to another centre and subsequent loss to follow up. By reviewing the medical notes of the remaining 48 patients we collected data on indications for, and timing of balloon atrial septostomy and subsequent complications and long term outcome. Results: The median time to follow up was 9.5 years (0.7–23.7). The diagnosis group was heterogeneous with 38 (79%) patients diagnosed with TA and normally related great arteries with or without pulmonary stenosis or atresia. The remaining 10 patients had more complex anatomy. Of the 48 patients, 5 (10%) were deemed to require balloon atrial septostomy for a clinically and/or echocardiographically restrictive atrial septum. Three procedures were performed within the first 24 hrs of life and 2 within the first month. One of the patients developed NEC following the septostomy and subsequently died of overwhelming sepsis. One patient required delayed surgical septectomy at 6 years of age, during a bidirectional Glenn operation. Of the remaining 42 patients there were 7 deaths (16%) but none were attributed to a restrictive atrial septum (1–at birth, severe acidosis and multi organ failure due to interrupted aortic arch and poor response to IV Prostin; 1–5 days with Group B streptococcal sepsis; 1–post operative sepsis following insertion of a BT shunt; 1–9 years, acute shunt obstruction; 1–withdrawal of care due to complex anatomy deemed to have a poor prognosis; 2-sudden death at home at 3½ years and 11 years). 26 patients required palliation with an arterial shunt (19–modified BT shunt, 6–Waterston Shunt, 1–ductal stent). None of the patients developed a clinically and/or echocardiographically restrictive atrial septum post procedure or during long term follow-up. Conclusions: We conclude that elective balloon atrial septostomy at presentation may not be necessary in patients born with tricuspid atresia. Accepting the limitations of retrospective data collection we also conclude that progressive narrowing of the interatrial septum at a later date is an unusual occurrence, even after an arterial shunt operation. We recommend that atrial septostomy should only be performed in patients who show obvious clinical and/or echocardiographic evidence of restrictive interatrial communication.

Covered Stent Treatment of Right Pulmonary Artery Stenosis and Waterston Shunt

A 35-year-old man with residual Waterston shunt and concomitant right pulmonary artery stenosis after mutiple surgery for tetralogy of Fallot presented with significant left to right shunt and left heart failure. We describe the percutaneous placement of an endovascular stent graft to occlude the Waterston shunt and relieve the right pulmonary artery stenosis simultaneously. This novel use of a covered stent effectively treated a residual problem without reoperative thoracotomy.

Transposition of the great vessels manifested on radionuclide ventriculography.

Ventricular ejection fraction was evaluated in a 30-year-old man. At birth he underwent a Rashkind procedure and placement of a Waterston shunt, followed by a Mustard procedure at age 2 years. In the Mustard procedure, an oblique right atriotomy is created, the atrial septum is excised, a pericardial baffle is sutured to incorporate the pulmonary veins into the right atrium, and another baffle is sutured to direct superior vena cava and inferior vena cava return into the left atrium and mitral valve. Radionuclide ventriculography showed that the systemic ventricle was the enlarged anatomic right ventricle. The systemic atrium is the anatomic right atrium and the pulmonary atrium is the anatomic left atrium. The ejection fraction of the right ventricle was estimated to be 32%.

Cross sectional and Doppler echocardiographic evaluation of aortopulmonary shunts.

Shunt vessels were imaged and shunt flow was analysed by cross sectional and Doppler echocardiography in 12 patients who had had 14 shunt procedures (nine left Blalock-Taussig shunts, three right Blalock-Taussig shunts, one modified Waterston shunt, and one central shunt). The shunt vessels were classified by echocardiography as uniformly patent, segmentally stenosed, and uniformly stenosed. These findings were compared with those of angiography. Also the peak flow velocities at the aortic and the pulmonary ends of the shunt vessels were measured by Doppler echocardiography and the ratio of these values was calculated for each shunt. Twelve (85.7%) of 14 shunt vessels were imaged along their entire length by cross sectional echocardiography. The two remaining shunt vessels were only partially imaged. In 10 patients who also had angiography the echocardiographic and angiographic images of the shunt vessels were identical. The ratio of the peak flow velocity measured at the aortic and the pulmonary ends of the shunt vessel was significantly larger in the segmentally stenosed shunt vessels than in the uniformly patent shunt vessels (p < 0.001). The ratio in the two shunt vessels only partially imaged by cross sectional echocardiography indicated that they were segmentally stenosed. The combination of cross sectional and Doppler echocardiography may be useful for determining either the patency or the morphology of an aortopulmonary shunt.

Early and Late Results of the Modified Waterston Shunt with PTFE Grafts for Palliation of Complex Congenital Cyanotic Heart Disease in Neonates

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)

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.