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Parachute valve is the malformation of an atrioventricular valve in which the tension apparatus springs from a single papillary muscle or group of muscles. Parachute tricuspid valve is a rare anomaly with no surgically repaired case to date. We describe a case of parachute deformity of the tricuspid valve leading to hemodynamically significant severe tricuspid stenosis. The present case was successfully repaired surgically along with atrial septal defect (ASD) and ventricular septal defect (VSD) closure., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

This three-part article examines the histologic and morphologic basis for stenotic and purely regurgitant tricuspid valves. In Part I, conditions producing tricuspid valve stenosis are reviewed. In over 90% of stenotic tricuspid valves, the etiology is rheumatic disease. In isolated tricuspid stenosis, the etiology is either carcinoid or congenital. Rare causes of tricuspid stenosis include active infective endocarditis, metabolic or enzymatic abnormalities (Fabry's, Whipple's disease), and giant blood cysts.

A new approach to creating an interatrial communication using a stationary fixed-diameter angioplasty balloon is presented. Stationary balloon septostomy was used to create an atrial communication in 19 young swine using a transseptal technique through a closed atrial septum, and in 18 newborn lambs through a patent foramen ovale. In animals with an intact septum, there was persistent patency of the atrial communication using balloons larger than 12 mm in diameter. The average diameter of the atrial communication was 96% of the expanded diameter of the angioplasty balloon. For newborn lambs with a patent foramen, a smooth oval atrial communication was noted by 4 weeks, and there was persistent patency of the opening using balloons larger than 15 mm in diameter. The average diameter of the opening was 81% of the diameter of the angioplasty balloon used. The new procedure was used successfully in five patients. All five showed resolution of the clinical symptomology. In conclusion, stationary angioplasty balloon atrial septostomy has been effective in producing interatrial communications, which remained patent for 4-14 weeks, in animals with intact septum, as well as in those with a patent foramen ovale.

Patients with cyanotic congenital heart disease who have obstruction proximal to the pulmonary arteries and, hence, do not develop pulmonary hypertension, can go through pregnancy successfully, if the pregnancy and delivery are adequately cared for, with possible complications in mind. We report a successful pregnancy in a woman with congenital tricuspid stenosis and a patent oval foramen.

Isolated congenital tricuspid valve stenosis is a rare entity. The modalities of definitive treatment have been surgical repair or replacement. We present the first reported patient with congenital tricuspid stenosis successfully treated by percutaneous balloon valvotomy. This appears to be an alternative to surgery when the anatomy, as demonstrated by echocardiography, is suitable.

A rare case of congenital tricuspid stenosis treated with modified Fontan operation was reported. The patient was 13 years old boy and his chief complaint was cyanosis. Cyanosis appeared in his neonatal period and squatting developed in his childhood. He underwent Blalock's shunt operation at 5 years of age. His exercise tolerance was limited at the admission for the radical operation. Angiocardiography showed the annular stenosis of tricuspid valve and the moderate hypoplasia of right ventricle. The right ventricular volume was 56 ml (equivalent to 35% of the normally expected right ventricular volume) and the diameter of tricuspid annulus was 13 mm (equivalent to 58% of the normally expected diameter). Atrial septal defect was associated with but pulmonary stenosis did not exist. His left ventricular function was good and pulmonary vascular resistance was not raised. A diagnosis of tricuspid stenosis was made and Fontan's repair was considered to be possible for his correction. Operative findings showed normal architecture of tricuspid valve with the three leaflets but tricuspid annulus was narrow. Tricuspid valve stenosis was considered to be caused by the hypoplasia of tricuspid ring and surgical dilatation was found to be difficult. Therefore, modified Fontan operation (RA-RV connection) was performed. His cyanosis disappeared and exercise tolerance was improved after the operation. Cardiac catheterization and angiocardiography were performed 8 months and 28 months after the operation. His righ ventricular volume was increased to 123 ml (equivalent to 57% of the normally expected volume) followed by RA-RV connection.

The authors report the case of a child followed up from the age of 4 months to the age of 15 for asymptomatic congenital heart disease. Initial clinical examination showed the presence of a systolic murmur and a diastolic rumble in the xiphoid area with signs of right atrial dilatation-hypertrophy. During follow-up, an apical diastolic rumble was detected with left atrial dilatation from the age of 3. Doppler echocardiography carried out recently showed double mitral and tricuspid valve stenosis with two-dimensional appearances of doming of both valves and moderate transvalvular pressure gradients with slight elevation of pulmonary artery pressures on the Doppler study. The very early detection of the malformation and the continuous follow-up of this patient indicate the congenital and not rheumatic origin of this double valve stenosis.

Congenital tricuspid valve stenosis in the absence of other valvular abnormalities is rare. In this report we describe a patient with congenital tricuspid valve stenosis, ostium secundum atrial septal defect, and electrocardiographic left anterior fascicular block, who presented with paradoxical emboli. This case, as well as previous case reports, suggests that congenital tricuspid stenosis with ostium secundum atrial septal defect is associated with left anterior fascicular block.

Percutaneous balloon valvuloplasty was successfully performed in a 55-year-old Chinese woman with severe recurrent tricuspid and pulmonic stenoses 11 1/2 years following open valvotomies. An Inoue balloon catheter with the balloon made of double-layered rubber and nylon micromesh was used. The orifices of the tricuspid and pulmonic valves increased from 13 to 27 mm in diameter and from 6 to 14 mm in diameter, respectively, following concurrent balloon valvuloplasties. The present report demonstrated for the first time the feasibility of percutaneous balloon valvuloplasty for combined valvular stenoses.

Balloon valvuloplasty has already gained its place as an alternative option in the treatment of valvar stenoses. In congenital valvar stenosis its application will be limited because of the complexity of the underlying valve deformities. Nevertheless, isolated pulmonary and aortic valve stenosis are amenable to this form of treatment. The major limitations are undeveloped arterial valve rings and valve dysplasia. In acquired valvar stenosis the main limitation of balloon valvuloplasty seems to be massive calcification of the affected valves. Basically, in both the arterial and atrioventricular position, balloon valvuloplasty splits fused commissures and fractures calcific deposits. Excessive radial pressures may cause tears of the leaflets. The potential of thromboembolic complications, therefore, warrants caution. The nature of the damage inflicted on pathologic valves almost by necessity dictates that restenosis will occur and - most likely - with a high incidence.

In a 13-day-old, 1,300 gram premature infant with tricuspid atresia, a Potts-Smith anastomosis was successfully performed. We used a neurosurgical vessel clip, smaller than the smallest available vascular clamp, to side-clamp the descending aorta. This report appears to be the first of the construction of a Potts-Smith shunt in an infant of this size.

The incidence of congenital heart disease (C.H.D.) in Liverpool from 1960 to 1969 inclusive has been determined from the Liverpool Congenital Abnormalities Registry with a follow-up period of 3 to 12 years. The incidence is 6-6 per 1000 total births and this probably represents a very small degree of under-reporting. There is no consistent seasonal variation in the incidence of any of the main congenital heart lesions. In general, infants with C.H.D. tend to be of lower birth weight and born after shorter gestation than controls. This is most conspicuous with patent ductus arteriosus (P.D.A.). Females preponderate in P.D.A. and males in transposition. There is probably also a male preponderance in coarctation and aortic stenosis. Fallot's tetralogy is associated with increased maternal age and parity. Pregnancies leading to the birth of a baby with C.H.D. are complicated by threatened abortion more frequently than are controls. The concordance rate for C.H.D. in twins is low. Monozygotic twins are more liable to C.H.D. than are dizygotic twins. The incidence of C.H.D. in the siblings of affected propositi is 2-3 times that expected. Affected sibs often have the same lesion. About 20 per cent of infants with C.H.D. have associated major defects notably monogolism and defects of the alimentary, skeletal, genito-urinary and nervous systems. These are responsible for the early death of about one quarter of all infants born with C.H.D. The data presented here suggest that environmental rather than genetic factors are predominantly responsible for congenital heart disease.

The case is presented of a patient with congenital tricuspid stenosis who was treated successfully by a palliative open operation. The preoperative diagnosis was tricuspid atresia. At operation, however, congenital tricuspid stenosis was detected and managed by commissurotomy.. The postoperative course was very good. Later, the associated ventricular septal defect (VSD), pulmonary annular stenosis, and probably the atrial septal defect (ASD) will be corrected. We emphasize the importance of diagnosing congenital tricuspid stenosis correctly, because of the possibility of operating upon the valve itself.

The clinical profile and course of 101 patients with tricuspid atresia seen at the Children's Hospital Medical Center, Boston, were reviewed; the electrocardiograms, hemodynamic data, associated anomalies, complications of the malformation and surgical results were outlined and a definition of anatomic types and radiologic groups formed in order to facilitate therapeutic decisions. Overall survival to 15 years of age was approximately 50 percent. Tricuspid atresia type 1 (normally related great arteries), group A (decreased pulmonary vascularity) was the most frequent category (54 percent); without surgery, these patients had only a 10 percent chance of survival beyond the 1st year of life. Surgical intervention improved chance of survival to 15 years of age to 50 percent. Overall surgical mortality was 23 percent. The Waterston shunt is the procedure of choice in symptomatic small infants with diminished pulmonary flow (group A). Type II patients with increased pulmonary blood flow (group B) would benefit from a pulmonary arterial band. In contrast, patients with group B, type I atresia would rarely need a pulmonary arterial band. Patients with spontaneously changing hemodynamics (group C) usually require a shunt later in life than those in group A, with more favorable operative results. Our experience indicates the continued need of early surgical palliation. New surgical approaches, such as the right atrial-pulmonary arterial anastomosis, may result in a more prolonged survival.

Fourteen patients underwent a physiological operation for tricuspid atresia. Seven patients survived up to 3 years after operation. Six patients underwent hemodynamic studies 2 to 34 months postoperatively (mean, 15 months). Average age at the time of operation was 14 years (range, 5 to 25 years). Postoperative studies showed 4 patients to be in sinus rhythm and 2 in junctional rhythm. Right atrial pressure was elevated an average of 17 mm Hg (range, 10 to 34 mm Hg). All patients showed good atrial transport function regardless of their rhythm. Two had a right atrium-left pulmonary artery gradient of 1.5 to 10 mm Hg across the conduit. Average arterial saturation was 92% (range, 87 to 97%), on improvement of 13% over preoperative values. Residual hypoexmia was due to pulmonary vein desaturation and to atrial right-to-left shunting early postoperatively and later, to atrial right-to-left shunting alone.

We report a case of congenital triple valve stenosis with dominant pulmonary valve disease, sparing the aortic valve, presenting at the age of 51 years with progressive shortness of breath and central cyanosis. At post mortem, no intracardiac right to left communication could be demonstrated.

This article will review the qualitative and quantitative methods of radionuclide evaluation of intracardiac shunts. Radionuclide angiocardiography of the cardiopulmonary system has proven adequate, with few exceptions, for qualitative detection of left-to-right and right-to-left intracardiac shunts. Numerous authors have reported on the detection of intracardiac shunts using qualitative methods such as rapid sequential visualization of nuclide angiocardiography using the scintillation camera with Polaroid, 35mm or 70mm film. Recent development of videotape storage systems and computers offers a quantitative technique of permanently recording the nuclide angiocardiogram and thus the capability for replay and analysis of the study. Quantitative analysis of the nuclide angiogram in the form of heart chamber dilution curves or pulmonary dilution curves permits 1) determination of the presence or absence of left-to-right, right-to-left, or bi-directional intracardiac shunts, including shunts which might go undetected using the qualitative techniques, 2) shunt location at the atrial or ventricular level, 3) determination of the functional status of a corrective left-to-right shunt, and 4) shunt quantitation. By quantitative analysis of the isotope dilution curves, it is possible to calculate the size of left-to-right or right-to-left intracardiac shunts (Qp/Qs ratio) to within 10% of the values obtained at cardiac catheterization.

Echocardiograms were obtained simultaneously with the standard indocyanine green injections performed during cardiac catheterization. When injected into cardiac chambers, indocyanine green produced a cloud of echoes that enabled us to visualize characteristic flow patterns in the presence of normal and abnormal cardiac hemodynamics. More than 300 echocardiographic contrast studies have been recorded in patients with various congenital and acquired cardiac defects. The patterns obtained were reproducible, sensitive, and specific.

Total correction of underdeveloped right ventricle related to tricuspid hypoplasia has been performed in 6 patients at the department of thoracic and cardiovascular surgery University Göttingen. In 5 patients reconstruction of a four-chamber-four-valved heart could be accomplished. 1 patient with a poor result after Fontan's operation, performed one year previously, showed a significant growth in size of the hypoplastic right ventricle in the postoperative angiogram, but died 14 hours after reoperation. Prior to corrective surgery, 4 patients required 1 or 2 palliative procedures: 3 Glenn- and 2 Blalock-Taussig operations and a pulmonary valvulotomy in 1 patient. A Hancock-valved conduit was inserted between the right atrium and the hypoplastic right ventricle as an inlet of this chamber after closure of the ASD and VSD in 4 patients. The conduits are bypassing the hypoplastic tricuspid valve orifices closed during corrective surgery. In the remainder 2 patients with moderate tricuspid and right ventricle hypoplasia, closure of the ASD was performed only. Anomalous muscle bundles and fibrous tissue inside the right ventricular cavity were resected surgically, safely. 4 to 12 months after corrective surgery the 5 survivors were investigated by cardiac catheterization. In the 3 patients operated with a valved conduit there was a moderate growth in size of the hypoplastic right ventricle, the right atrial pressure was elevated and ranged from p = 14 to 16 mm Hg. There was a slight right atrial-right ventricular gradient of 4 mm Hg across the valved conduit. The 2 patients with closure of the ASD and VSD only had a nearly normal-sized right ventricle and a right atrial pressure of p = 10 mm Hg or less at the postoperative right ventricle and a right atrial pressure of p = 10 mm Hg or less at the postoperative investigation. All of them were acyanotic and had an improved or normal exercise tolerance. We concluded that reconstruction of an underdeveloped right ventricle in well-selected cases will yield more physiological benefits than do current surgical procedures, in which a conduit is interposed between the right atrium and the main pulmonary artery.

A case of tricuspid atresia by sealing of the valves, associated with an intact ventricular septum, a hypoplastic right ventricle and the absence of the pulmonary valve, is described. The foramen ovale and the ductus arteriosus were widely patent but there was no interventricular communication. The pulmonary flow was assured by the ductus which had a saccular continuation in the pulmonary trunk. It is postulated that the lesions probably occurred during the period between the XIX and XXII horizons and that the malformed right coronary artery may have played an important role in producing the lesions of the right ventricle.

We present a patient with severe tricuspid stenosis whom at the age of 13 developed a brain abscess successfully treated by surgical means.

Twelve patients with tricuspid atresia underwent physiological operative repair. The criteria for their selection for operation included normal pulmonary vascular resistance and normal left atrial and left ventricular end-diastolic pressures. Four patients died (30% mortality). The 8 surviving patients developed pleural effusion, ascites, and hepatomegaly, which markedly improved in the subsequent weeks. Five of the 8 survivors underwent cardiac catheterization. The arterial oxygen saturation in these patients averaged 82% preoperatively, 89% immediately postoperatively, and 94% or better six months later. All had improved subjectively and developed increased exercise tolerance.

A case is presented illustrating delayed failure of a right Glenn anastomosis due to patency of the superior vena cava-right atrial (SVC-RA) junction. Clinical deterioration was rectified by a unique nonoperative approach. A transvenous catheter was designed, constructed, and used to accurately place an umbrella-like occlusive wafer at the SVC-RA junction. In this manner the integrity of the cavopulmonary shunt was re-established. A gratifying and uncomplicated palliation has thus far been achieved.

Congenital tricuspid stenosis is an uncommon congenital anomaly and usually associated with other lesions such as severe pulmonic stenosis or atresia. Sometimes, it is difficult to diagnose this malformation non-invasively, but the differential diagnosis from tricuspid atresia is essential for the surgical considerations. Described here were three patients with congenital tricuspid stenosis who were successfully diagnosed by contrast echoangiography using peripheral venous injection of the contrast medium. Contrast echoes appeared in the right atrium initially and then in the right ventricle through the stenotic tricuspid valve. In conclusion, contrast echoangiography is mandatory in the diagnosis of this entity.

In addition to recording the motion of the mitral, tricuspid, aortic, and pulmonic valves, echocardiography can identify right and left ventricular cavities and the interventricular septum. Disorders such as atrial-septal defect, valvular and subvalvular aortic stenosis, pulmonic stenosis, Ebstein's anomaly of the tricuspid valve, and the hypoplastic left-heart syndrome can readily be evaluated by echocardiography. In tetralogy of Fallot and truncus arteriosus, discontinuity between the anterior aortic wall and septum with overriding aorta has been demonstrated. Doubleoutlet right ventricle is associated with posterior aortic wall and mitral valve discontinuity. In disorders such as single ventricle, tricuspid atresia, and endocardial cushion defect with common A-V canal, echocardiographic demonstration of the absence of the interventricular septum has provided the clinician with valuable information. Newer techniques such as compound-B ultrasonography, which produces a two-dimensional cross-sectional image of intracardiac structures, and multiscan echocardiography will enhance the use of conventional echocardiography by providing a more accurate anatomic display of cardiac chambers and outflow vessels.