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Whilst the pulmonary valve is a tricuspid valve, very few reports exist of bicuspid pulmonary valves, the majority of which are associated with congenital heart disease. Isolated bicuspid valves not associated with congenital abnormalities are even more rare. Herein, the case is described of a 65-year-old man who died from a stroke, and in whom a bicuspid pulmonary valve was found during post-mortem dissection for tissue donation.
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A balloon-valvuloplasty of the pulmonary valve (BVP) was carried out in 14 patients within the first 40 days of life. Six infants had a critical valvular pulmonary stenosis with intact ventricular septum (group S), 5 infants had a pulmonary atresia with intact ventricular septum (group A), and 3 infants had a tetralogy of Fallot (group F). In 4 of the 5 patients of group A the BVP was preceded by operative opening of the pulmonary valve. In one case radiofrequency perforation of the valve during the first diagnostic cardiac catheterization could be carried out successfully. The average value of the ratio of the balloon diameter to the diameter of the pulmonary valve was 1.31 +/- 0.20. The systolic pressure in the right ventricle dropped 51 mm Hg (from 106 +/- 32 mm Hg to 55 +/- 9 mm Hg) in group S and 33 mm Hg (from 101 +/- 27 mm Hg to 68 +/- 19 mm Hg) in group A. In group F the arterial oxygen saturation increased 15%, however the pressure of the right ventricle was, after the BVP, slightly higher than before. The average residual gradients after BVP were in group S 28 +/- 15 mm Hg, in group A 34 +/- 12 mm Hg and in group F 48 +/- 18 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: Pulmonary valve incompetence is usually well tolerated after tetralogy of Fallot repair but may result in late progressive right heart failure as manifested by increasing fatigue, dyspnea, and frequently arrhythmias. METHODS: All patients who underwent pulmonary valve replacement in our center late after repair of tetralogy of Fallot were reviewed. RESULTS: Eighty-five patients had elective pulmonary valve replacement late (median, 9.3 years) after repair. Operative risk was low (1.1%). Ninety percent of survivors are in New York Heart Association class I. Survival 10 years after pulmonary valve replacement is 95%, with 86% of the patients free of reoperation for valve failure. CONCLUSIONS: Pulmonary valve replacement is infrequently required after repair of tetralogy of Fallot. Pulmonary valve replacement may be performed electively with little risk; it improves symptoms of right heart failure and provides satisfactory long-term survival with low risk of early valve failure. As the population of patients who have had repair of tetralogy of Fallot ages, pulmonary valve replacement will become a more frequent consideration.
OBJECTIVE: The aim of this study was to identify the long-term character of the domestic bileaflet mechanical valve in the chronic implanted sheep model and to evaluate the potential value of the modal. METHODS: Six adult sheep underwent implanted mechanical bileaflet valve in pulmonary position under the cardio-pulmonary bypass with beating heart. The chronic implanted sheep model was built up and observed in the respects of a long-term survival, function of prosthesis and pathological specimen. RESULTS: Six adult sheep survived with good condition after operation. The average survival period of six sheep was (221 +/- 208) days. Two sheep were postoperatively sacrificed in 41 and 71 days, respectively. The necropsy revealed normal valve function without thrombosis, periprosthetic leakage and overgrowth of fibrous tissue. One sheep died from dysfunction of prosthetic valve at the postoperative 196 days. The reason was the prosthetic thrombosis with slight overgrowth of fibrous tissue in periprosthesis. The other two sheep died from severe anemia at the postoperative 196 days and 234 days, and the autopsy revealed no abnormal finding else. And one remained to survive with good condition up to now (over 617 days) and was checked by Doppler echocardiogram twice at the postoperative 438 days and 479 days, respectively. The results showed normal function of the bileaflet valve in pulmonary position. CONCLUSION: The long-term good effects would be achieved by using the implanted new domestic bileaflet valve in pulmonary position of sheep.
Tetralogy of Fallot with absent pulmonary valve has been classified to a few groups. The most severe one is characterized by symptomatic onset immediately after birth. The others are no or slightly symptomatic at least during their neonatal period. In a severely symptomatic 12-day-old neonate of tetralogy of Fallot with absent pulmonary valve who had required intubation immediately after birth, tight pulmonary banding and left side modified Blalock-Taussig shunt were performed on emergency basis. Consequently, prior massive pulmonary regurgitation was decreased significantly. Forty-five days after this first stage operation, he weaned from respiratory management. At 1-year-old, radical repair based on conotruncal repair, which consisted of patch closure of ventricular septal defect preserving the tricuspid septal leaflet function, resection of anterior wall of enlarged left pulmonary artery, and right ventricular outflow tract reconstruction using autologous tissue and a pericardial patch was performed. Bicuspid pulmonary valve, posterior one of procured autologous pulmonary wall and anterior one of polytetrafluoroethylene (PTFE) respectively, was created to minimize deterioration of the pulmonary insufficiency. Although postoperative cardiac function was kept feasible showing his central venous pressure of 7 mmHg in the main, postoperative general course was eventful especially regarding the respiratory function. The patient was weaned from the prolonged ventilator management 5 months after this radical repair eventually. Generally, to diminish the massive pulmonary regurgitation in early lifetime period could reduce a progressive airway obstruction and minimize pulmonary tissue damage. However, even after the total correction in this case, considerable peripheral segmental pulmonary obstructive lesions were persistent according to the perfusion lung scanning with 99mTc macroaggregated albumin and 99mTechnegas ventilation lung scanning studies. This persistent, supposed to be innate, pulmonary obstructive lesions might prevent ordinal recovery after cardiac radical repair for this most severe subtype of absent pulmonary valve syndrome.
Absent pulmonary valve is a rare cardiac malformation, usually associated with a tetralogy of Fallot. It features aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory distress. The experience of our group over a 20 year period (20 patients) is reported. The diagnosis was usually made in the neonatal period in the presence of cardiomegaly and a systolic and diastolic murmur at the left sternal border. The diagnosis was made at antenatal echocardiography in 2 cases. Ten patients were not operated and 4 of them died in the neonatal period of respiratory distress and 2 others of extracardiac causes. Three children are on the waiting list for surgical correction and the remaining child is asymptomatic. The other 10 cases were operated with a minimum closure of the ventricular septal defect and widening of the pulmonary tract. The latter procedure was performed with an infundibulo-pulmonary patch (6 cases) and insertion of a pulmonary valve (4 patients) (monocusp patch in 3 cases, pulmonary heterograft in one case). Eight patients underwent a reduction procedure of the pulmonary arteries to relieve branchial compression. Of those who were operated, two died during follow-up: the first of complete atrioventricular block and the second of infectious endocarditis of the stenosed pulmonary heterograft. These results show that reduction procedures of the pulmonary arteries are promising approaches which should improve the future prognosis of young patients with respiratory distress. The need for insertion of a pulmonary valve remains debatable.
The relation between the size of the balloon used for dilatation of the pulmonary valve and the extent of relief of pulmonary stenosis both immediately after and at intermediate term follow up was studied. Sixty four balloon dilatation procedures in 56 patients were divided into group 1-12 in which the ratio of the diameter of the balloon to that of the pulmonary annulus was less than or equal to 1.0--and group 2-52 in which the ratio was greater than 1.0. Both groups had similar mean (SD) pressure drops across the pulmonary valve before dilatation. Immediately after dilatation there was a significant reduction in the pulmonary valve gradient in both group 1 (84.3 (39.2) v 43.6 (26.8) mm Hg) and group 2 (92.8 (42.1) v 22.4 (13.6)mm Hg). On intermediate term follow up (6-34 months), residual pulmonary valve gradients were significantly lower in group 2 (20.8 (18.5)mm Hg) than in group 1 (75.0 (49.4) mm Hg), suggesting that restenosis was more common after dilatation with small balloons. These data suggest that although the immediate results with either small or large balloons are good, balloons that are larger than the pulmonary valve annulus produce more sustained relief of pulmonary stenosis. Further analysis within the group treated with larger balloons showed that the subgroup with a balloon/annulus ratio of 1.01-1.2 had more recurrences of stenosis (need for repeat balloon dilatation and larger number of patients with residual pulmonary valve gradients greater than 30 mm Hg) than subgroups with balloon/annulus ratios for diameters of 1.21-1.41, greater than 1.41, and greater than 1.5, in which there were no recurrences. Balloons that were > 1.5 times the size of the pulmonary valve annulus had no additional advantage over the other subgroups, namely, 1.21-- 1.4 and > 1.41. These results and reports of damage to the right ventricular outflow tract by oversized (> 1.5) balloons indicate that balloons that give a balloon/annulus ratio 1.2 -- 1.5 are the best for dilatation of the pulmonary valve.
A 1-day-old asymptomatic neonate with a to and fro precordial murmur was diagnosed by cross sectional echocardiography to have Fallot's tetralogy with absent pulmonary valve, and origin of the left pulmonary artery from the ascending aorta. Moderate stenoses at the origin of the anomalous left pulmonary artery and of the right pulmonary artery were present, allowing definitive surgical correction to be deferred.
Partial anomalous pulmonary venous drainage (PAPVD) with an intact atrial septum is a very rare cardiovascular anomaly. Most PAPVD cases can be detected by echocardiographic scanning of the anomalous pulmonary venous connection. This paper describes an asymptomatic 6-year-old boy who was referred because of a heart murmur. An electrocardiogram and two-dimensional echocardiography revealed right heart volume overload. Mild pulmonary valve stenosis and an intact atrial septum were noted. In addition, color flow mapping demonstrated the right upper pulmonary vein connecting to the superior vena cava. This paper emphasizes that two-dimensional echocardiography in conjunction with color flow mapping is a valuable modality in the detection of anomalous pulmonary venous drainage.
BACKGROUND: Congenital absence of the pulmonary valve syndrome (APVS) is a rare cardiac defect characterized by aneurysmal pulmonary arteries, hypoplastic pulmonary valve cusps, and tracheobronchial abnormalities. Absence of the pulmonary valve syndrome usually occurs in conjunction with ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO). Surgical mortality rates as high as 16% to 56% have been reported. Here, we describe the surgical results using the Lecompte maneuver, reduction pulmonary arterioplasty, and a valved right ventricle to pulmonary artery (RV-PA) conduit. METHODS: Medical records and operative and echocardiography reports for all surgical APVS cases were retrospectively examined for pertinent clinical variables. A patient with left bronchial compression due to enlarged pulmonary arteries associated with totally anomalous pulmonary venous drainage (TAPVD) is included to illustrate the value of the Lecompte maneuver. RESULTS: From January 2002 to December 2004, 4 children with APVS had surgery at a median age of 5 months (range, 3 months to 3.5 years). Three had malalignment VSD and RVOTO. Four had respiratory signs (cough, wheeze, tachypnea, oxygen dependence, ventilator dependence), and all 5 had evidence of tracheobronchial compression by computed tomography or magnetic resonance imaging. Repair included a Lecompte maneuver, a valved conduit with reduction pulmonary arterioplasty, and VSD closure as necessary. The TAPVD patient had repair of the anomalous veins and a Lecompte maneuver. There have been no deaths, and all patients were discharged in good condition. Follow-up is complete at a median of 24 months (range, 9 to 35). Echocardiography has shown no progressive enlargement of the pulmonary arteries. CONCLUSIONS: Use of the Lecompte maneuver, reduction arterioplasty, and a valved conduit for repair of APVS provides favorable early and midterm results.
A 74-year-old man was admitted to our hospital complaining of dyspnea. Parasternal transthoracic echocardiography showed a quadricuspid pulmonary valve above the aortic valve and a hypoplastic accessory cusp between the right and left cusps of the pulmonary valve. The pulmonary valve ring diameter was normal (26 mm) but the transvalvular peak velocity was 3.5 m/sec, suggesting a pressure gradient of 49 mmHg across the pulmonary valve. The pulmonary valve had thickening and decrease in mobility of the leaflets without complete closure during diastole, and severe pulmonary regurgitation was present. Heart failure was treated successfully with digitalis and diuretics. Quadricuspid pulmonary valve is difficult to identify using transthoracic echocardiography because of the anatomical features. In this case, the dilated main pulmonary artery caused the pulmonary valve orifice to shift anteriorly, allowing visualization of the short-axis view of the pulmonary valve.
The absent pulmonary valve syndrome consists of a severely hypoplastic pulmonary valve with anular stenosis, aneurysmal dilatation of the main pulmonary artery with one or both pulmonary branches also dilated, and a ventricular septal defect. From 1955 to 1975, 15 patients were seen at Texas Children's Hospital with this syndrome. Although anatomically similar to tetralogy of Fallot, the most significant symptoms during early infancy were secondary to bronchial compression resulting from the dilated pulmonary arteries and enlarged left atrium. Intracardiac repair performed on two infants at four months of age failed to ameliorate the respiratory symptoms and both died. Seven patients from 2.3 to 16 years of age were operated upon, with one late postoperative death. Five other patients have not yet received surgical intervention. We recommend vigorous continual respiratory therapy for for infants and small children and intracardiac repair in older children since repair in infancy does not relieve respiratory symptoms. We do not recommend a prosthetic pulmonary valve unless there is persistently elevated main pulmonary artery pressure.
Surgical treatment of neonates with pulmonary atresia and intact ventricular septum is associated with a high morbidity and mortality. During the last two years specific radiofrequency-catheters have been developed, which allow the interventional perforation and subsequent balloon dilatation of an atretic pulmonary valve during cardiac catheterization. We report our experience with this new interventional technique in two neonates. In the first case perforation and balloon dilatation of the atretic pulmonary valve was performed without a complication. In the second case the radiofrequency catheter perforated the right ventricular outflow tract. There was no pericardial tamponade and the patient underwent a surgical valvotomy the following day. In our experience radiofrequency-assisted balloon dilatation of the pulmonary valve is a possible alternative in the treatment of neonates with pulmonary atresia, intact ventricular septum and moderate hypoplasia of the right ventricle. Further experience will have to show, whether this new interventional technique can be generally recommended.
A 6 month old female child with tetralogy of Fallot with absent pulmonary valve leaflets and anomalous origin of left pulmonary artery was admitted to hospital with a mild adenoviral lower respiratory tract infection. She had a large haemoptysis 1 week after admission, and recovered with simple supportive measures. This case illustrates a previously unreported complication of a rare anomaly.
Pulmonary valve endocarditis is an uncommon disease. Improved visualization of the pulmonary valve on the longitudinal views provided by biplane transesophageal echocardiography (TEE) improves the diagnostic utility of TEE in pulmonary valve endocarditis. We report three cases of isolated pulmonary valve endocarditis diagnosed with biplanar TEE.
Doming of the pulmonary valve is considered an important angiographic sign for valvar stenosis. But, this sign cannot be used with similar specificity following valvotomy because at the time of surgery a bicuspid valve is created. Pre- and postoperative hemodynamic data and right ventriculograms are reviewed in 22 patients with simple pulmonary valvar stenosis. There was no correlation between the transvalvar gradient and appearance of the pulmonary valve after surgery.
In 3 patients with absent pulmonary valve syndrome and absent ductus arteriosus, the lungs were injected and analyzed postmortem using morphometric techniques. Two patients had tetralogy of Fallot and 1 had D-transposition of the great arteries, the latter being the first autopsy-proved case of absent pulmonary valve with transposition. In addition to the expected dilatation of the central pulmonary arteries and compression of the mainstem bronchi, postmortem pulmonary arteriography revealed a bizarre pattern of hilar branching. Instead of single segmental arteries, tufts of arteries arose which entwined and compressed the intrapulmonary bronchi. In all 3 patients the histologic structure of the pulmonary arteries was abnormal. The elastic lamina of the media of the right and left pulmonary arteries were increased in number outside the lung, but were decreased within the lung. At both sites, the elastic laminae were thickened and fragmented. In the 2 ventilator-dependent patients, there was slight medial hypertrophy and extension of muscle into normally nonmuscular arteries. In 1 of the 2 cases in which the number of bronchial generations was counted, they were decreased, and in the 1 case in which bronchial count was unknown, alveolar multiplication was severely impaired. Therefore, our data may explain why, in some patients with absent pulmonary valve syndrome, relief of compression of the mainstem bronchi alone does not appreciably alleviate or reverse severe respiratory disease.