Pulmonary Valvotomy Research Articles

Perioperative considerations in a sickle cell patient undergoing cardiopulmonary bypass.

An 11-year-old child, a known case of sickle cell anaemia with a history suggestive of sickling crisis in the past was scheduled for surgical pulmonary valvotomy. Pre-operative blood transfusion and hydroxyurea were administered. Pre-operative blood transfusion is indicated in sickle cell disease patients to raise the haematocrit level and lower sickle haemoglobin (HbS) levels. Before the start of cardiopulmonary bypass (CPB), exchange transfusion was performed to reduce HbS level and raise adult haemoglobin level. Hypothermia was prevented by employing normothermic CPB.

From Atretic to Perforated: A Closer View of Pulmonary Atresia

A 7-day-old male infant with membranous pulmonary atresia and intact ventricular septum (PA/IVS) underwent hybrid pulmonary valvotomy. Access was via median sternotomy guided by intra-operative epicardial ultrasonography. After puncturing the atretic pulmonary valve, a 6mm Wanda balloon catheter was inflated to dilate the atretic valve. Adequate valve movement with antegrade flow was confirmed using real time epicardial echocardiography. Intra-operative epicardial ultrasonography is an effective tool for guiding PA/IVS during hybrid procedures. Epicardial echo imaging provides clear images with excellent quality, helps guide the hybrid operation, and prevents the need for the ionizing radiation of fluoroscopy.

Catheter-based Treatment in Patients with Critical Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum: A Single Institute Experience with Comparison between Patients with and without Additional Procedure for Pulmonary Flow

We report a single institute experience of transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by a systemic-pulmonary shunt in patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). In addition, we compare patients with or without an additional source of flow to support the pulmonary circulation after successful pulmonary valvotomy. All neonates with PAIVS or CPS who underwent primary transcatheter pulmonary valvotomy between January 2004 and December 2010 were reviewed retrospectively. Some of them needed an additional source of flow to support the pulmonary circulation. We performed a comparison between those who required an additional source of pulmonary flow and those who did not. The initial procedure was successful in 20 out of 22 patients (seven of nine with PAIVS; all of 13 with CPS), but 10 of them needed an additional source of flow to support the pulmonary circulation: nine had arterial duct stenting and one had surgical Blalock-Taussig shunt. There were no deaths or major acute complications, except for femoral artery occlusion in three patients. The bipartite right ventricular morphology, the tricuspid z-score of ≤-0.74, the tricuspid to mitral valve ratio of ≤ 0.9, and the z-score of the diastolic interventricular septal thickness ≥ 2.37 in preprocedural examination showed more tendency of needing shunt placement. Transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by the arterial duct stent implantation was an effective approach in those patients. The angiographic distinction between CPS and PAIVS did not affect anything in this study including the procedural method, success, and odds for reintervention. The degree of right ventricle cavity hypoplasia provided the main restriction to forward flow after pulmonary valvotomy.

Porcine Bioprosthetic Valve in the Pulmonary Position: Mid-Term Results in the Right Ventricular Outflow Tract Reconstruction

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors' experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18-64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7-48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6-132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries.

Asociación inusual en el síndrome de Brachmann-de Lange

Brachmann-de Lange syndrome is a low incidence multiple congenital anomaly syndrome that includes a wide range of congenital heart diseases. We present a patient diagnosed with aortic coarctation during adulthood, who was subjected to surgical closure of ductus arteriosus and pulmonary valvotomy during the neonatal period. To our knowledge the combination of patent ductus arteriosus, pulmonary stenosis, and aortic coarctation in Brachmann-de Lange syndrome has not been previously described.

Double-chambered right ventricle in adults: an ‘uncommon’ entity, new ways of imaging

A 40-year-old man with previous cardiac surgery was referred to the adult congenital heart disease department at our hospital because of progressive dyspnoea during the previous 3 months. He had had successful pulmonary valvotomy when he was 9 because of a congenital pulmonary stenosis. As the initial approach in a patient with suspected heart failure (HF), a transthoracic echocardiography was performed in which a high-pressure gradient was measured by Doppler …

Minimally Invasive Approach for Mitral Valve Repair in a Patient With Prior Pentalogy of Fallot Repair

A 54-year-old woman with a history of pentalogy of Fallot with a right-sided aortic arch underwent a classic left Blalock shunt as a newborn and ventricular septal defect closure, resection of subvalvular right ventricular outflow tract obstruction, and pulmonary valvulotomy at 2 years of age. She subsequently had a transcatheter device closure of her atrial septal defect 8 years ago. She now presents with symptomatic severe mitral insufficiency caused by a flail posterior leaflet. Consistent with the patient’s diagnosis of pentalogy of Fallot, this patient has dextroposition and anterior displacement of the aorta, thus making sternal reentry perilous and compounding her operative risk for reoperative mitral valve surgery. 1 The dextroposition of the aorta and presence of an atrial septal device were also likely to limit exposure of the mitral valve through a sternotomy and preclude the use of a transseptal approach.

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct-dependent Pulmonary Circulation

Ductal stent (DS) in duct-dependent pulmonary circulation is less morbid than neonatal Blalock-Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. This is a retrospective review of clinical follow-up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Among 22 infants, four Group A patients followed for 26-54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short-term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5-14 months at a body weight of 5-7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6-8.5 kg after 8-15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow-up for hypoxia. Four patients had sudden death. The short-term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.

Use of a telescopic system for transcatheter radiofrequency perforation and balloon valvotomy in infants with pulmonary atresia and intact ventricular septum

Pulmonary atresia and intact ventricular septum is a complex congenital heart disease with great morphological variability. Approximately two-thirds of patients may be suitable for transcatheter pulmonary valvotomy. We reviewed our experience in the use of two different percutaneous approaches to evaluate the impact on fluoroscopy time and morbidity of a new technique to perform transcatheter radiofrequency perforation and valvotomy in newborns with pulmonary atresia and intact ventricular septum. In all, 31 patients underwent radiofrequency perforation of the pulmonary valve. The first 14 infants were treated using a 5 French Judkins right coronary catheter, which was manoeuvred directly underneath the atretic pulmonary valve (Group A). The others were treated using a telescopic system consisting of Northstar Lumax Flex and White Lumax Guiding Catheters (Cook; Group B). In both groups, after radiofrequency perforation of the pulmonary valve, a 0.014-inch superfloppy guidewire was advanced into the descending aorta and balloon dilations were performed. Required fluoroscopy time was significantly lower in Group B (48.5 ± 28.1 versus 24.9 ± 14.4 minutes, respectively; p < 0.01). A higher incidence of unfavourable events including the need for early surgery was found in Group A. In our experience, telescopic catheter proved to be a valid option able to decrease the fluoroscopy time of percutaneous radiofrequency perforation of pulmonary valve and consequently patients’ exposure to procedure-related risks.

An unusual fistula closed by percutaneous coil embolisation

A 25-year-old male patient was admitted for assessment of increasing shortness of breath and decreased transcutaneous oxygen saturation (91 %). He had a complex congenital heart defect, involving a dysplastic monocusp tricuspid valve and pulmonary valve atresia. The latter was palliated shortly after birth by a Brock procedure (entailing infundibular resection and pulmonary valvotomy). An atrial septal defect was surgically closed when he was 6 years old and a homograft was inserted in the right ventricular outflow tract at the age of 15. Replacement by a second homograft at the age of 23 was necessary together with tricuspid valvoplasty because of severe insufficiency of both valves. Apart from moderate pulmonary homograft stenosis (PHS) and re-occurrence of moderate to severe tricuspid insufficiency (TI) resulting in right heart failure, a right-left shunt was detected on transoesophageal echocardiography during intravenous agitated saline bubble-contrast injection, although the repaired atrial septum was intact. Invasive evaluation confirmed moderate to severe TI and a peak-to-peak gradient of 40 mmHg over the homograft. Right atrial pressures were above 20 mmHg. Contrast injection in the superior vena cava showed a very tortuous fistulous connection originating from the left axillary vein and draining towards the left atrium (Fig. 1, panel A and B). It was decided to close the fistula percutaneously and 4 coils (3 x IMWCE-3-PDA3 and 1 x IMWCE-3-PDA5, COOK MEDICAL, Bloomington, USA) were placed in the fistula, effectively occluding the vessel (panel C). The transcutaneous oxygen saturation increased slightly. Further management regarding the TI and PHS will be discussed with one option being percutaneous placement of a pulmonary valve (Medtronic, Minneapolis, USA), possibly in combination with another transcatheter valve prosthesis in tricuspid position to lower the right heart filling pressures. Fig. 1 a Contrast injection in the superior vena cava (SVC) showing a fistula (arrow) originating from the left axillary vein (AV). The right atrium (RA) is grossly enlarged (LIV left innominate vein). b Selective contrast injection in the fistula (arrow) reveals ...

Erişkinde Konjenital Aort ve Pulmoner Stenozu Cerrahi Onarımı

Although aortic and pulmonary valve stenosis are among the most common congenital heart defects, the combination of both aortic and pulmonary valve stenosis in the same patient appears to be very uncommon. Accurate diagnosis of combined valvular stenosis is imperative prior to surgical correction otherwise surgery of only one of the lesions may result in an insufficient hemodynamic improvement. A 34-year-old man with congenital aortic and pulmonary valve stenosis underwent Nick’s operation, aortic valve replacement, pulmonary valvotomy and resection of anomalous muscle of the right ventricular outflow tract. The operation was successfully performed and postoperative course was uneventful.

SELECTIVE PULMONARY VASODILATATION IMPROVES LEFT VENTRICULAR-RIGHT VENTRICULAR COUPLING IN PATIENTS WITH SIGNIFICANT PULMONARY VALVE REGURGITATION

Repair of tetralogy of Fallot (TOF) and pulmonary valvotomy for pulmonary stenosis (PS) often results in progressive pulmonary insufficiency (PI) and right ventricular (RV) dysfunction. The latter process has also been shown to hinder left ventricular (LV) function. We sought to examine whether

CAN SELECTIVE PULMONARY VASODILATOR THERAPY BE USED TO TARGET PULMONIC REGURGITATION? RESULTS OF THE PINOT NOIR TRIAL

Both tetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonic stenosis (PS) result in progressive pulmonary insufficiency (PI), which eventually necessitates repeat surgery. Though percutaneous therapy is growing in popularity, it remains ill-suited for native right ventricular (RV)

Surgical Decision Making in Neonatal Ebstein’s Anomaly

There is currently no consensus of opinion regarding the optimal surgical management of Ebstein's anomaly (EA) in neonates and young infants. Reported early mortality rates range from 25% to 100%. In this study, we present an algorithm for choosing the best management option for neonates with EA based on analysis of our experience. From 1994 to June, 2011, 48 neonates with a diagnosis of EA were managed by the same surgical team. Of these, two died before intervention; the remaining 46 either were managed medically initially (n = 20) or underwent surgical intervention during the neonatal period (n = 26) or early infancy (n = 9). The mean weight was 3.6 ± 1.7 kg (1.9-8.6) and mean follow-up time was 6.3 ± 4.5 years (0.2-16). Of the 20 patients initially managed medically, 11 remain well without intervention and nine required complete repair in infancy, with 100% survival. Of the 26 neonatal operations, 23 (88%) were complete biventricular repairs, 1 Starnes' palliation, and two Blalock-Taussig shunts (BTSs) ± pulmonary valvotomy. Among those having a two-ventricle repair, anatomic pulmonary atresia (APA) was a risk factor for early mortality (46.1%, 6 of 13) compared with those without pulmonary atresia (EA/no-PA; 10%, 1 of 10), P < .05. Most symptomatic neonates with EA will require early operation. For those with APA and mild tricuspid regurgitation (TR), a modified BTS and reduction atrioplasty may be the best initial option. For those with functional pulmonary atresia and severe TR and pulmonary regurgitation, ligation of the main pulmonary artery and placement of a BTS may provide the best initial palliation. For the rest, either a biventricular repair or a single-ventricle palliation is indicated.

Historical Aspects of Transcatheter Treatment of Heart Disease in Children

The very first transcatheter intervention to treat congenital cardiac defects was reported by Rubio-Alvarez et al. [1] in 1953, when they performed pulmonary valvotomy using a modified ureteral catheter. A decade later Dotter, Rashkind, Porstmann and their associates described progressive dilatation of peripheral arterial stenotic lesions, balloon atrial septostomy and transcatheter occlusion of patent ductus arteriosus, respectively. The purpose of this review is to present these and other historical developments of catheter-based interventions in the treatment of heart disease in children. Historical aspects of 1. balloon angioplasty/valvuloplasty of valvar pulmonary stenosis, valvar aortic stenosis, fixed subaortic stenosis, native aortic coarctation, postsurgical aortic recoarctation, branch pulmonary artery stenosis, mitral stenosis, cyanotic heart defects with pulmonary oligemia, stenotic bioprosthetic valves, congenital tricuspid and mitral stenosis, truncal valve stenosis, subvalvar pulmonary stenosis, supravalvar pulmonary stenosis (congenital membranous or postoperative), stenosis of the aorta (Leriche syndrome, atherosclerotic and Takayasu’s arteritis), baffle obstruction following Mustard or Senning procedure (both systemic and pulmonary venous obstructions), superior and inferior vena caval obstructions, pulmonary vein stenosis, pulmonary veno‑occlusive disease, vertical vein stenosis in total anomalous pulmonary venous connection, pulmonary venous obstruction following repair of total anomalous pulmonary venous obstruction, specially designed pulmonary artery bands, cor triatriatum, cor triatriatum dexter, and coronary artery stenotic lesions that develop after Kawasaki disease; 2. stents to enlarge stenotic lesions of branch pulmonary arteries, systemic veins, systemic and pulmonary venous pathways after Mustard procedure, aorta, right ventricular outflow conduits, pulmonary veins and native right ventricular outflow tract or to keep the ductus arteriosus open in patients with pulmonary atresia and hypoplastic left heart syndrome and maintaining patency of stenosed aorto-pulmonary collateral vessels, surgically created but obstructed shunts or acutely thrombosed shunts as well as covered stents; 3. transcatheter occlusion of cardiac defects comprising of atrial septal defect, patent foramen ovale, patent ductus arteriosus, ventricular septal defect and aortopulmonary window and 4. catheter-based atrial septostomy such as Rashkind balloon atrial septostomy, Park’s blade atrial septostomy, balloon angioplasty of the atrial septum, trans-septal puncture and atrial septal stents were presented.

Percutaneous Balloon Pulmonary Valvuloplasty

Congenital pulmonary valve stenosis is a common congenital heart disease. Isolated pulmonary valve stenosis comprises 8-10% of all congenital heart disease. While surgical pulmonary valvotomy has been available as a treatment since 1956, it requires a median sternotomy, use of cardiopulmonary bypass, and post-surgical ICU admission with multi-day hospitalization. Recognizing the potential advantages of a less invasive approach, the first attempts at percutaneous catheter-based dilation of stenotic pulmonary valves were performed in the 1950s. As the technique was refined and catheter and balloon technology have advanced, the results of balloon pulmonary valvuloplasty have improved and the approach has become the standard of care for treating pulmonary valve stenosis. The purpose of this article is to discuss the anatomy and physiology of pulmonary valve stenosis, describe the techniques and current technology, and review the outcomes of balloon pulmonary valvuloplasty.

Characterization of radiation exposure and effect of a radiation monitoring policy in a large volume pediatric cardiac catheterization lab

This study aimed to characterize radiation dose during cardiac catheterization in congenital heart disease and to assess changes in dose after the introduction of a radiation monitoring policy. Minimizing radiation exposure is an important patient safety initiative and relatively few data are available characterizing radiation dose for the broad spectrum of congenital cardiac catheter-based interventions. Radiation dose data were reviewed on all cases since 7/1/05 at a single large center. Procedures were classified according to 20 common case types then subdivided into five age categories. Groups with <20 cases were excluded. Radiation dose was estimated by cumulative air KERMA (mGy) and DAP (dose area product, μGym(2)) which were reported as median and interquartile range (IQR). We also examined differences in radiation dose before and after the implementation of a radiation policy. Between 7/1/05 and 12/10/08, 3,365 cases were identified for inclusion. Radiation dose increased with age and procedural complexity. Patients were characterized into low, medium, and high dose categories relative to each other. "Low" dose cases included isolated pulmonary or aortic valvotomy, pre-Fontan assessment, and ASD closure. "High" dose cases involved multiple procedures in pulmonary arteries or veins. After introduction of a radiation policy, there was a significant decrease in radiation dose across a variety of case types, particularly among infants and young children. Radiation dose in congenital cardiac catheterization varies by age and procedure type. A radiation monitoring and notification policy may have contributed to reduced radiation dose.

Estudo experimental do implante transcateter de prótese valvada autoexpansível na via de saída do ventrículo direito em porcos

Patients with congenital heart disease who underwent pulmonary valvotomy or surgery to open the pulmonary valve ring are prone to develop residual pulmonary insufficiency or stenosis that may lead to right heart failure with clinical deterioration. These children require multiple interventions throughout their lives, which impose a high rate of morbidity and mortality. To develop a less invasive technique for implantation of a valved prosthesis through the right ventricle. The valved prosthesis consists of an auto expanding metal stent built with nitinol, surrounded with polyester, where the three leaflets of bovine pericardium were mounted. Twelve pigs were used to perform the implants. Echocardiographic control was performed immediately after implantation and one, four, eight and 12 weeks. One animal showed reflux of moderate to severe and three mild reflux. Transvalvular gradients measured before implantation ranged from 3 to 6 mmHg and that soon after the implant was increased, ranging from 7 to 45 mmHg. There was a decrease in these gradients during follow up and in only four of the twelve animals the gradients were above 20 mmHg. Thrombus formation occurred in the prosthesis of six animals, and this was the most frequent complication. These findings highlight the need for studies with the use of anticoagulants and antiplatelet, an attempt to reduce this event. The study aims to contribute for the start of the use of prosthetic heart valves that could be implanted through minimally invasive techniques without the use of cardiopulmonary bypass.

Critical Pulmonary Stenosis

A 20-day-old girl was diagnosed with critical pulmonary valvular stenosis with patent ductus arteriosus (PDA). She underwent surgical pulmonary valvotomy and infundibular resection. A trial snaring of the PDA resulted in significant systemic desaturation, and the PDA was left undivided. A continuous infusion of prostaglandin was used to keep the PDA open for the next 8 days. The PDA acted as a "natural systemic-to-pulmonary shunt" to provide pulmonary blood flow until right ventricular compliance and function improved. The various causes of persistent desaturation following pulmonary valvotomy are discussed.

Management for neonatal pulmonary atresia with intact ventricular septum

Objective A retrospective study was undertaken to evaluate the outcomes of the newborns with pulmonary atresia with intact ventricular septum(PAIVS),in order to delineate strategies for the optimal management of PAIVS. Methods From July 2003 to July 2008,17 neonates with PAIVS underwent surgical treatment.The mean age at operation was(1 5.6±8.6)d and the mean weight was(3.54±0.44)kg.Initial surgical treatment included:Blalock-Taussig shunt (BT shunt)in 2 cases,closed pulmonary valvotomy and BT shunt in 1 case,right ventricular outlet tract reconstruction(RVOTR)in 2 cases,RVOTR and BT shunt in 11 cases. Results The mean duration of mechanical ventilation and intensive care unit stay were(35.5±35.1)h and(8.7±5.9)d,respectively.Two early deaths were reported inside the hospital(2/17,11.8%).Multivariable analysis demonstrated that mechanical ventilation before surgery was the risk factor for in-hospital mortality(r=1.02,P<0.01).The rest 15 cases were followed up with the average time of 19 months(2 months to 5 years)and no death was reported. Conclusions Neonates with PAIVS should be operated early.Individualized treatment strategy and regular follow-up are helpful to achieve better short-term outcome. Key words: Pulmonary atresia; Cardiac surgical procedures; Heart defects,congenital; Infant,newborn