Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair"

BACKGROUND: Between 1982 and 1984, we successfully performed "one and a half ventricular repair" using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. METHODS: In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years post-operatively. RESULTS: All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. CONCLUSIONS: Although the lower limits of the tricuspid valve diameter for "one and a half ventricular repair" using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.     

Clinical results of the staged Fontan procedure in high-risk patients

BACKGROUND: For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared. METHODS: Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (> or =20 mm Hg), high pulmonary vascular resistance (> or =3 Wood units), small pulmonary artery (Nakata index <200 mm2/m2), atrioventricular valve incompetence (> or = moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same period, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group). RESULTS: In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group). CONCLUSIONS: A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.     

Intrapericardial Blalock-Taussig shunt

The standard Blalock-Taussig (B-T) shunt is preferred over other shunts in the treatment of infants with cyanotic heart disease and reduced pulmonary blood flow. The B-T shunt is not, however, without problems such as phrenic nerve injury and excessive bleeding. It is a time-consuming procedure owing to the degree of lateral mediastinal dissection required (the latter is especially important in critically hypoxic infants). An alternative procedure is described in which an intrapericardial approach is used. This approach requires minimal dissection of the subclavian artery and the anastomosis is performed to the pulmonary artery, medial to the superior vena cava (SVC) and lateral to the aorta. The procedure was performed on 17 infants from 1 day to 2 years of age (average age of 9.4 months, with seven of the 17 infants being less than 6 months of age). Follow-up ranged from 1 to 44 months (average of 17 months). In 94%, the shunt was functioning well. The potential advantages of this approach include: elimination of phrenic nerve injury, easy dissection with reduced operating time, reduced intraoperative hemorrhage, and perhaps a larger anastomosis with improved patency rate.     

Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan patients

BACKGROUND: A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation. METHODS: Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 +/- 257 days). RESULTS: Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p = 0.01). Pulmonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly for 1.8 +/- 1.1 to 0.7 +/- 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them. CONCLUSIONS: The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.     

Arterial prosthesis of microporous expanded polytetrafluoroethylene for construction of aorta-pulmonary shunts

A new arterial prosthesis made of polytetrafluoroethylene (OTFE) was evaluated in 10 infants with complex cyanotic congenital heart disease. All grafts used were 4 mm. in diameter and varied in length from 0.8 to 6 cm. The grafts were anastomosed to the main pulmonary artery or its bifurcation in 8 infants and to the right and left pulmonary arteries in one each. There were two early deaths, one of which was related to shunt failure. The advantages of a shunt to the main pulmonary artery are obvious, and the intraoperative procedure is facilitated with the prosthesis. Follow-up averages 9 months in the 8 survivors, and the patients have nearly doubled their preoperative weight. A shunt murmur is present in each case and the children have mild-to-moderate cyanosis at rest. Repeat aortograms in 2 patients, 8 and 10 months postoperatively, show a smooth graft without luminal narrowing. The aortic oxygen saturations were 73 per cent. The PTFE graft and/or anastomoses will not grow with the growth of the child and therefore may not accommodate growth by increased flow. This may prove to be a limiting factor in its long-term use in fants. We reserve the use of these grafts for infants with complex cyanotic defects undergoing emergency surgery or in older children in whom a conventional shunt is not possible or a previous shunt has failed.     

A case of total cavopulmonary connection with preceding tricuspid valve closure in pulmonary atresia and intact ventricular septum, right ventricular-coronary artery fistulae, and coronary artery stenoses]

More than half of the patients with pulmonary atresia and intact ventricular septum (PA/IVS) are known to complicate right ventricular-coronary artery fistula (fistulae) and particularly those with coronary artery stenoses bear a high mortality rate and remain in a surgical challenge. A 4-year-old girl was first admitted to our hospital at 5 days of age and right ventriculogram revealed markedly hypoplastic tripartite ventricle and multiple fistulae through which both coronary arteries and aortic root were retrogradely opacified. Echocardiographically measured diameter of the tricuspid valve was 5 mm (Z value: 4). She underwent pulmonary valvotomy and central aorto-pulmonary (AP) shunt at 16 days of age. Repeat right ventriculogram at 1.9 years of age disclosed multiple stenoses in left anterior descending coronary artery (LAD) with proximal dilatation and tortuosity. Additional findings of left ventricular dysfunction (LVEF of 61.5%) and depressed ST segment in left precordial leads prompted us to proceed to the second palliation which comprised take-down of central AP shunt, bidirectional cavopulmonary shunt and closure of tricuspid and pulmonary valves. Catheterization at 3.4 years of age disclosed antegradely filled LAD with apparent relief of stenoses and improvement of LVEF to 68.9%. She underwent definitive repair of total cavopulmonary connection at 4.0 years of age and is leading a normal life 2 years after surgery. This experience draws us to conclude that tricuspid valve closure is a meaningful palliative procedure for PA/IVS with fistulae and coronary artery stenoses, provided that proximal segments of both coronary arteries remain intact.     

A chemoenzymatic synthesis of UDP-(2-deoxy-2-fluoro)-galactose and evaluation of its interaction with galactosyltransferase

The left internal mammary artery-to-the left pulmonary artery shunt was created in a 16-year-old boy with single ventricle, severe pulmonary stenosis palliated by Glenn shunt at the age of two. Four years follow-up angiogram demonstrated a significant increase of the diameter of the left internal mammary artery from 4 to 7 mm. The internal mammary artery is a good alternative conduit for a systemic-to-pulmonary artery shunt for a cyanotic heart disease because of its growth potential.     

Total cavopulmonary connection for grown diminutive pulmonary artery after staged Blalock-Taussig shunt

We report a two-year-old girl with asplenia, [A, L, L] DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm2/m2. Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm2/mm2, but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm2/m2, right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering necessity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.     

Squamotacin: an annonaceous acetogenin with cytotoxic selectivity for the human prostate tumor cell line (PC-3)

A 12-year-old child with tricuspid atresia and acquired hypoplasia of the left pulmonary artery was successfully treated with unilateral Fontan operation. Angiography at age 2 months had shown a normal left pulmonary artery, and a modified Potts shunt was performed. An emergency central shunt was required a year later. Reinvestigation 5 years after the initial operation revealed severe hypoplasia of the left pulmonary artery.     

Pulmonary artery growth after systemic-to-pulmonary shunt in children with a univentricular heart and a hypoplastic pulmonary artery bed. Implications for Fontan surgery

The aim of the study was to investigate the developmental pattern of hypoplastic pulmonary artery (p.a.) bed augmented by systemic-to-pulmonary shunt in children with univentricular heart scheduled for Fontan surgery. For the study, a highly selected patient cohort was chosen (12 patients aged between 5 and 19 years; mean 9.5 years) with comparable initial morphological conditions of univentricular heart and hypoplastic p.a. bed, who after mandatory systemic-to-pulmonary shunt underwent Fontan procedure at time of normalization of pulmonary artery size. Further selection criteria were: normal pulmonary vascular resistance at time of Fontan procedure, competent a-v valve(s), and globally unimpaired ventricular function. All patients were grouped according to the preoperative pulmonary flow index (Qpi; L/min/m2 b.s.a.) measured immediately before Fontan operation: Group A: 1.5-2.5; B: 3.0-4.0; C: 4.0-5.0; D: > 6.0, and their cardio-pulmonary hemodynamic situation (Hb, SAsat%, Qp/Qs, PAP, Rp/Rs, EDVP, FS%, ventricular diastolic compliance (VC = EDVP/Qpi + Qsi) as well as the pulmonary artery size and area using standard (Nakata-index, McGoon-ratio) and a self designed computer assisted planimetric area calculation (PPAAI; cm2/m2 b.s.a.) analysed. Each patient underwent 1-3 shunt procedures, the mean shunt patency period for groups A, B, C and D was 12, 8.6, 5.3, and 4.5 years, respectively. The mean Nakata-index (283, 297, 324, 405 in groups A-D) and the McGoon-ratio (2.0, 2.2, 2.8, 3.3 in groups A-D) correlated with the Qp index, reflecting flow dependent development of pulmonary artery bed. No correlation was found between Qpi and PPAAI (47, 40, 41 and 47 in group A-D). The VC/Qp relation showed an inversely proportional pattern with values 2.3, 1.0, 0.8, 0.7 for corresponding groups A-D, the lowest VC in group A correlated with polyglobulic status (Hb- values; g/dl): 21.3 in A vs 19.8, 18.0 and 16.5 in B-D) and mean arterial SAsat-values (77% in A vs 83%, 84% and 89% in B-D). In conclusion, in our highly selected patient cohort, the development of p.a. size was strongly flow-dependent, and patients with restrictive pulmonary flow needed an approximately threefold longer time period to normalize their p.a. size compared to those with excessive flow. In patients with restrictive pulmonary flow, the Nakata-index underestimated the degree of development of the pulmonary artery system, probably due to the distortion of the proximal p.a. segment. In consequence, in these patients the normalization of the p.a. bed and thus suitability for the Fontan procedure probably occurred much earlier. Based on our observations and those of others, in patients with excessive flow the normalization of p.a. bed, provided it occurs within 3-4 years, seems not necessarily to be associated with a deterioration of ventricular function.     

Recanalisation of an occluded modified Blalock-Taussig shunt by balloon dilatation

A four year old boy with pulmonary atresia and ventricular septal defect had an acute cyanotic episode three years after undergoing a right-sided, 6 mm diameter, modified Blalock-Taussig shunt. On admission no continuous murmur could be heard from the shunt and the typical high velocity, continuous flow profile of the shunt could not be identified by Doppler echocardiography. At catheterisation a right subclavian artery angiogram confirmed shunt occlusion. From the subclavian artery, an 0.035 inch wire was used to enter the occluded shunt and then the pulmonary artery. Balloon angioplasty of the entire length of the shunt was performed with 6 mm diameter balloon. After angioplasty the arterial oxygen saturation increased from 63% to 83%. The patient was treated with intravenous heparin followed by warfarin. Repeat catheterisation and angiography eight days later confirmed wide patency of the shunt.     

Effectiveness of an umbilical artery "snare assisted" approach for critical pulmonary valve stenosis or atresia in the neonate

Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.     

Primary bidirectional superior cavopulmonary shunt in infants between 1 and 4 months of age

The performance of a primary bidirectional superior cavopulmonary shunt procedure in early infancy is attractive because it minimizes the number of operations needed before a Fontan procedure, avoids ventricular volume overload and its sequelae, and eliminates pulmonary artery distortion. However, concerns over elevated or labile pulmonary vascular resistance have limited its use in the first few months of life. Nine patients aged 1 to 4 months (5 patients, < 2 months) have undergone a primary bidirectional superior cavopulmonary shunt procedure between October 1992 and March 1994. Primary diagnoses were tricuspid atresia (n = 4), asplenia syndrome (n = 2), polysplenia syndrome (n = 1), double-outlet right ventricle (n = 1), and double-inlet left ventricle (n = 1). Associated lesions of immediate surgical importance were total anomalous pulmonary veins (n = 2), a restrictive atrial septum (n = 4), bilateral superior venae cavae (n = 5), and patent ductus arteriosus (n = 5). The surgical procedure consisted of unilateral (n = 4) or bilateral (n = 5) bidirectional superior cavopulmonary shunt and the repair of associated lesions. Of significance, in 4 of our first 5 patients a very limited additional source of pulmonary blood flow was provided because of a low arterial oxygen tension immediately after cardiopulmonary bypass. Pleural effusions developed in 2 of these 4 patients. In subsequent patients cardiopulmonary bypass was not used whenever possible or, if it was needed, use of an extra source of pulmonary blood flow was avoided.(ABSTRACT TRUNCATED AT 250 WORDS)     

Destructive gouty arthritis of the hip

We present a case of a 19-year old female with systemic pulmonary artery (PA) pressure due to a congenital ventricular septal defect (VSD) and atrial septal defect (ASD). She was pink at rest and cyanotic on exercise. Lung biopsy revealed grade IV pulmonary vascular changes. As a preliminary step PA was banded to increase right-to-left shunt and decrease aortic (Ao) saturation with consequent decrease in PA saturation. After one year, when she was no longer cyanotic, even on exercise, lung biopsy revealed total regression of pulmonary vascular changes. As a definitive procedure VSD and ASD were closed and PA was debanded. Cardiac catheterization one week postoperatively showed PA pressure to be 50% of systemic pressure. We postulate that reversal of pulmonary vascular changes were due to lowered PA saturation. We further believe that lower PA pressure could have contributed to this regression of pulmonary vascular changes. We performed the same procedure in six more patients with similar positive clinical response. This new concept brings renewed hope to many children who otherwise are candidates for heart lung transplantation.     

Effect of pulmonary microembolism on arteriovenous shunt flow

The effects of acute pulmonary hypertension on the fraction of cardiac output shunted through pulmonary arteriovenous communications have been studied in dogs as a possible cause of hypoxia following pulmonary embolization. Pulmonary artery pressure was increased twofold and then fourfold above control values by embolization of the pulmonary vascular bed with polystyrene microspheres. Quantitative measurements of arteriovenous shunt were determined from the fraction of 50 mu radioactively labeled microspheres injected into the inferior vena cava which passed through the pulmonary circulation into systemic vascular beds. There was no increase in the fraction of pulmonary blood flow passing through pulmonary arteriovenous connections, 50 mu in diameter or greater, with pulmonary microembolism when FIo2 was 1. There was a small increase in arteriovenous shunt fraction when pulmonary artery pressure was increased with an FIo2 of 0.21. Physiological shunt measured by the oxygen technique did not increase with pulmonary embolism, but total venous admixture rose significantly. Postmortem gravimetric measurements of lung water indicated pulmonary edema. We conclude that anatomic arteriovenous shunt channels have little physiological significance after pulmonary microembolism in the dog lung. The major cause of hypoxia immediately after pulmonary microembolism is ventilation/perfusion imbalance, probably caused by pulmonary edema.     

A new operative technique for pulmonary artery banding: adjustment of pulmonary artery bands by mitral valve flow velocity

We developed a new technique to adjust the pulmonary artery band at surgery by monitoring the mitral valve flow velocity, which is indirectly indicative of the pulmonary flow. We employed this technique for 10 consecutive patients aged from 5 days to 5 months (mean, 1 months) weighing from 2.7 to 4.4 kg (mean, 3.3 kg). Underlying disease was aortic coarctation or interrupted+ventricular septal defect in 7 patients, single ventricule in 1 patients and miscellaneous defects in 2 patients. The pulmonary artery was exposed through a left lateral thoracotomy and a 3 mm wide Teflon tape was placed around the main pulmonary artery. The transducer of the Doppler echocardiography was placed along the left sternal border. The band was tightened gradually until the maximum velocity of the mitral valve flow decreased to around 70% of the previous level. During banding procedure, arterial oxygen saturation, heart rate and left ventricular contractility were monitored continuously. If bradycardia, unacceptable hypoxemia or ventricular dysfunction occurred, the band was released. The mitral valve flow velocity decreased rapidly by just a little additional tightness of the band between the range of 50% to 80% of the previous level. This technique enabled a very fine adjustment (less than 0.5 mm plication) and postoperative management has become very easy. Although there is a limitation of this technique that monitoring of the mitral valve flow velocity cannot be applied to the patients with significant interatrial shunt or mitral regurgitation, we conclude that this technique is simple and useful to obtain the optimum constriction of the pulmonary artery with excessive pulmonary blood flow.     

Balloon pulmonary valvuloplasty for infants with severe tetralogy of Fallot

Balloon pulmonary valvuloplasty was performed in 3 infants with severe tetralogy of Fallot at days 24, 54 and 86 because of progressive hypoxemia. In two patients, the balloon catheter (4 cm long, 5-8 mm diameter) could not pass through the pulmonary valve. This necessitated a smaller balloon and required a two-step procedure. Initially, a coronary artery balloon (2 cm long, 3.5 mm diameter) was used. Following balloon valvuloplasty, arterial oxygen saturation increased from 63 to 83% in case 1, from 69 to 85% in case 2 and 63 to 86% in case 3. Immediate postvalvuloplasty right ventricular cineangiography revealed that the maximal opening diameter of the pulmonary valve leaflets increased from 1-2 mm to 3-4 mm in cases 1 and 3, and from 2-3 mm to 4-5 mm in case 2. No significant complications occurred. Echocardiographic follow-up data showed that the diameter of the right ventricular outflow tract and pulmonary arteries increased with age. The present results show that the pulmonary valvuloplasty is an effective procedure for relief of pulmonary valve stenosis in tetralogy of Fallot and to improve oxygenation and growth of the pulmonary arteries and right ventricular outflow tract without the need of an immediate aortopulmonary shunt.     

Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS: Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described. RESULTS: There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome. CONCLUSIONS: Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic, pulmonary, or systemic and pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS AND RESULTS: Between March 1990 and March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation. CONCLUSIONS: Anomalous pulmonary venous return can significantly complicate the management of the single-ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients.     

Pulmonary regurgitation in large atrial shunts without pulmonary hypertension

Seven patients with pulmonary regurgitation (PR), normal pulmonary artery (PA) pressures and large left-to-right atrial shunts are reported. Six had secundum atrial septal defects (ASD) and one had anomalous pulmonary venous drainage. These comprised 4% of 180 patients with atrial shunts and normal PA pressures. Pulmonary regurgitation was diagnosed clinically by mid-frequency diastolic decrescendo murmurs beginning after the pulmonic component of the second heart sound, and diagnoses were confirmed by catheterization. In two patients who had serial preoperative catheterizations over 8 and 16 years, PR progressed in one and was present only on the second study in the other. All patients underwent shunt correction, at which time the pulmonic anulus and artery appeared dilated, but the pulmonic valves were normal and did not require revision. In all patients the PR murmur disappeared after shunt correction alone, and on chest X-ray both PA and overall heart size decreased. Although it is known that pulmonary regurgitation occurs with atrial septal defects and pulmonary hypertension, the present study demonstrates that it also occurs with high flow atrial shunts, in which setting it has different implications and is reversible with shunt correction alone.