Pulmonary atresia with ventricular septal defect in adults

BACKGROUND: Multistage surgery culminating in completed hemodynamic repair is now performed for pulmonary atresia with ventricular septal defect (PA-VSD). Justification for operation in patients with an adequate collateral pulmonary circulation is controversial. Data on natural adult survival are scant but are necessary to provide the rationale for multistage reconstructive procedures. METHODS AND RESULTS: All cyanotic adults with PA-VSD in the UCLA Adult Congenital Heart Disease Center Registry from 1978 through 1992 formed the basis for this study. Registry data and echocardiographic, hemodynamic, and angiographic information were used to determine longevity, clinical course, and operative feasibility. Of 26 patients, 16 were unoperated when referred (group A), and 10 had been palliated before age 18 years (group B). Two thirds were 18 to 29 years old. Only 2 patients survived beyond age 40 years. Six died during follow-up at a mean age of 31 years (+/- 12.1 SD). Eight group A patients were in New York Heart Association class II, and 8 were in class III. Of the 26 group A and B patients, 20 had aortic regurgitation, which was moderate or severe in 10. Eight had cardiac failure. Of 11 group A patients who remained unoperated, 5 died. Twelve patients were considered eligible for surgery at > or = 18 years of age. Ten underwent completed hemodynamic repair with a mean post-operative right ventricular-to-left ventricular systolic pressure ratio of 0.45 (+/- 0.16 SD), and there were no early or late deaths. CONCLUSIONS: Even when collateral blood flow permits adult survival, all such patients are symptomatic. Mean life expectancy without operation did not exceed three decades. Aortic regurgitation and cardiac failure are significant negative variables. Nearly half of unoperated adults died during follow-up. Staged hemodynamic repair can be performed with a low surgical risk in properly selected adults with PA/VSD.     

Acute aortic dissection and ventricular septal defect repair

A 37-year-old male with long-standing heart murmur and ventricular septal defect developed acute chest pain and was diagnosed with an aortic dissection and aortic insufficiency. The ventricular septal defect and aortic dissection were repaired successfully as a combined procedure. At late follow-up (three years), he continued to enjoy an excellent result.     

Surgical management of aortic regurgitation associated with ventricular septal defect

The intracarotid amobarbital procedure was carried out in 8 male and 7 female candidates to temporal lobectomy, and a female candidate to frontal lesionectomy, aged 18-50 (mean 32.5) years. Language and memory were tested after injection in each hemisphere. Both were measured by the Montreal procedure. In 9 patients language and memory were evaluated with the Seattle procedure too. In 12 patients the left hemisphere was dominant for language; three had bilateral dominance. In 1 patient the Seattle procedure demonstrated the dominant hemisphere by relatively slowness of speech during the drug effect in the left hemisphere. Memory was defined to be in the left hemisphere in 12 patients, in the right in 2, bilateral in 1 and in another lateralization was not possible. In 1 patient memory dominance was determined by the Montreal protocol alone because of lack of cooperation. These early results indicate that the methods may be complementary for determination of language and memory dominance in epilepsy surgery candidates.     

Pseudoaneurysm of the left ventricle following repair for ventricular septal perforation

The patient was a 64-year-old man who was treated surgically for an infarct-related ventricular septal perforation. Pseudoaneurysm of the left ventricle was recognized on the 38th postoperative day. Emergency surgery was performed. It seemed that insufficient resection of the infarcted myocardium was performed during the initial surgery to avoid narrowing the ventricular dimension by direct closure of the left ventricle, but this resulted in pseudoaneurysm of the left ventricle. Left ventricular free wall plasty with a patch should be performed during the initial surgery.     

Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect

OBJECTIVE: "Renal dose" dopamine is widely used in the perioperative period to provide renal protection. A comprehensive review of the literature was performed to determine whether dopamine does in fact confer protection on the kidneys of surgical patients. SUMMARY BACKGROUND DATA: Studies in healthy animals and human volunteers reveal that dopamine causes diuresis and natriuresis, as well as some degree of renal vasodilatation. RESULTS: Studies of the perioperative use of dopamine fail to demonstrate any benefit of dopamine in preventing renal failure. Studies in congestive heart failure, critical illness, and sepsis also fail to show any benefit of dopamine other than diuresis. Further, dopamine administration is not completely without risk, because of dopamine's catecholamine and neuroendocrine functions. CONCLUSIONS: Routine use of prophylactic "renal dose" dopamine in surgical patients is not recommended.     

Aortic valve prolapse and aortic regurgitation associated with subpulmonic ventricular septal defect

The natural development of aortic valve deformity was studied in 315 patients with subpulmonic ventricular septal defect. The patients with early development of aortic regurgitation had a pulmonary-to-systemic flow ratio of > 1.5, normal right ventricular pressure, and severe aortic regurgitation.     

Development of double-chambered right ventricle after surgical closure of a ventricular septal defect

The double-chambered right ventricle (DCRV) is a rare cardiac malformation, often associated with ventricular septal defect (VSD). CASE REPORT: The authors describe a case of DCRV in a child who underwent surgical repair of perimembranous VSD at the age of 7 months because of systemic pulmonary artery hypertension and growth retardation. At this time, no pressure gradient was measured within the right ventricle and no muscle proliferation was observed on echocardiography or right ventriculography. Postoperative follow-up revealed hypertrophy of the moderator band accompanied by progressive development of a right midventricular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 years. The child was operated after invasive confirmation of the diagnosis of DCRV and the haemodynamic data. The child is now 6 years old and no restenosis has been observed. CONCLUSION: DCRV is usually associated with VSD, but the course of the two diseases can be dissociated. The diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examination reveals a systolic ejection murmur.     

A reconstruction of the left ventricle for post infarction left ventricular aneurysm complicated by ventricular septal perforation--a case report

The aging of bitemarks through the subjective interpretation of visual signs has created an area of controversy in the forensic science community. The healing dynamics of these bite wounds has been insufficiently studied and is poorly understood. A review of the literature related to bitemark aging was undertaken in an effort to summarize the currently published information on this misunderstood subject.     

The management of severe subaortic stenosis, ventricular septal defect, and aortic arch obstruction in the neonate

Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.     

Right ventricular volume characteristics in ventricular septal defect

Right and left ventricular volume characteristics were determined from biplane cineangiocardiography in 37 patients with isolated ventricular septal defects. Patients were divided into three categories as determined by the degree of left-to-right shunt: small shunt-less than 35% of pulmonary blood flow (N=9); moderate shunt-35-49% (N=8), and large shunt-greater than 50% (N=20). Right ventricular (RV) end-diastolic volume was increased above normal in 15 of 20 studies performed in patients with large left-to-right shunts and averaged 159 +/- 10% of normal (P less than 0.001). In contrast, only one of the patients in the small shunt group and only half of the patients in the moderate shunt group showed increases in RV end-diastolic volume. The increase in RV volume was proportional to the corresponding increase in left ventricular end-diastolic volume, with the right ventricle ranging from 48 to 116% of LV end-diastolic volume (average 83%). Right ventricular ejection fraction was normal in all patient groups. Right ventricular outpur was increased commensurate with the increases in the RV end-diastolic volume. These data indicate that substantial augmentation in RV end-diastolic volume does occur in patients with isolated ventricular septal defects and large left-to-right shunts. These data can be explained by the significant diastolic and "isovolumic" shunting from left ventricle to right ventricle which occurs in these patients.     

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.     

Apical muscular ventricular septal defects between the left ventricle and the right ventricular infundibulum. Diagnostic and interventional considerations

BACKGROUND: Effective transcatheter or surgical closure of apical muscular ventricular septal defects (VSDs) requires accurate delineation of variable and often complex anatomy. These defects have generally been considered as communications between the apexes of both left and right ventricles. METHODS AND RESULTS: Among 50 consecutive patients with multiple muscular VSDs referred for transcatheter device closure between October 1987 and April 1993, a subset of 10 patients (aged 7 days to 28 years) with apical muscular VSDs shared a unique set of anatomic characteristics: (1) large and often single opening in the left ventricle; (2) multiple right ventricular openings in the anterior aspect of the apical septum; and (3) separation of the right ventricular apical region into which the VSDs open from the rest of the right ventricular inflow and outflow by prominent muscle bundles. Additional analysis of the anatomy by use of echocardiography and cineangiography showed that these muscular defects were between the left ventricular apex and right ventricular infundibular apex. In 6 patients, the transcatheter devices used to create a septum in these hearts were placed in the right ventricle, straddling muscle bundles that separated the apical VSD from the rest of the right ventricular inflow and outflow, resulting in incorporation of a portion of the right ventricular infundibular apex into the physiological left ventricle. Three patients had devices placed between the apexes of the left ventricle and the infundibulum. The defect closed spontaneously within the right ventricle in 1 patient. One patient died after surgery for tetralogy of Fallot in situs inversus. The remaining 9 patients were all clinically well at the time of their most recent follow-up visit (follow-up duration, 32 +/- 11 months). This distinct type of apical VSD was identified by echocardiography in 20 of 274 patients who were followed up clinically for muscular VSDs. CONCLUSIONS: Left ventricular-infundibular apical VSDs constitute a distinct morphological type of muscular VSD that can be distinguished by echocardiography and cineangiography. In selected cases, the infundibular apex can be separated from the rest of the right ventricular inflow and outflow to eliminate flow across these defects.     

Leaflet''s free edge suspension for correction of aortic insufficiency associated with ventricular septal defect

Antibody in sera from 560 cattle of tuberculosis (TB)-infected and TB-free herds was investigated by competition and indirect enzyme-linked immunosorbent assays using bovine purified protein derivative tuberculin as the antigen. Antibody was detected in sera from both types of herd, with a widely overlapping range of titers. However, a "tail" of high-titered sera was observed for the distribution of data for only those cattle from TB-infected herds.     

Aneurysm of the membranous ventricular septum with ventricular septal defect, mitral and tricuspid insufficiency

Bile acids are known to promote colon carcinogenesis. However, there is one study showing that ursodeoxycholic acid (UDCA) supplemented in the diet at the concentration of 0.4% prevented azoxymethane-induced rat colon tumorigenesis. The aim of our study was to explore the inhibitory effect of a much smaller dose of UDCA on colon carcinogenesis in rats. One hundred 7-week-old F344 rats were given 2 mg of N-methylnitrosourea 3 times a week for 3 weeks by intrarectal instillation, and were fed a 0% (control), 0.4% or 0.08% UDCA-supplemented diet for the next 27 weeks. All the rats were killed and examined for tumor development at week 30. The tumor incidence and number were significantly lower and smaller, respectively, in the UDCA-fed rats than in the control rats: 40% and 36% vs. 68%; 0.5 +/- 0.1 (mean +/- SEM) and 0.4 +/- 0.1 vs. 1.0 +/- 0.2. All the tumors were located in the distal half of the colon and were plaque-shaped or polypoid, being well-differentiated adenocarcinomas restricted to the mucosa or submucosa. Bile acids in the feces and the blood obtained at weeks 20 and 30, respectively, were analyzed by HPLC. A significant increase of UDCA was confirmed in both the feces and the blood of the UDCA-fed rats compared with the control rats. The results suggest that the continuous feeding of a small dose of UDCA may prevent colon carcinogenesis.     

Nonobstructing accessory mitral valve tissue and ventricular septal defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

Afterload reduction in the management of postinfarction ventricular septal defect

The primary goal in the medical management of ventricular septal defect complicating myocardial infarction is to support cardiac function and control symptoms, if possible, for a period of 4 to 6 weeks. If the patient survives this period, surgical correction of the defect is technically easier and safer. In many cases, However, cardiac function is severly compromised, intractable biventricular failure develops,early operation is necessary and the likelihood of successful repair is diminished.     

Relationships between left ventricular mass, left ventricular work and coronary artery size in aortic regurgitation--possible mechanism of myocardial ischemia

To investigate the relationships between coronary artery size, left ventricular (LV) mass, and LV stroke work in aortic regurgitation (AR), these values were measured in 19 patients with severe AR. Twenty normal subjects and 15 patients with mitral regurgitation (MR) were used as control groups. The coronary area index, i.e., the coronary cross-sectional area divided by body surface area (BSA), was larger in the AR group than in the control groups in all measured sites except for the peripheral left anterior descending coronary artery (LAD) and right coronary artery (RCA). However, the coronary area index divided by the LV mass was significantly smaller in AR and MR patients than in normal subjects. Furthermore, the coronary area index divided by LV stroke work was smaller in AR patients than in MR patients and normal subjects. These results suggest that the coronary blood flow associated with the increased LV mass and stroke work caused by regurgitation was insufficient in patients with severe AR, especially in the area of the LAD. Therefore, the occurrence of myocardial ischemia in patients with severe AR may involve inadequate enlargement of the coronary artery which perfuses the LV, in addition to factors such as decreased coronary perfusion pressure, increased coronary artery resistance and decreased coronary flow reserve.     

Performance of the lift ventricle in left ventricular obstructive cardiomyopathy

Whether volume expansion influences NaC1 reabsorption by the diluting segment of the nephron remains a matter of controversy. In the present studies this question has been examined in normal unanesthetized dogs, undergoing maximal water diuresis. Free water clearance (CH2O/GFR) has been used as the index of NaC1 reabsorption in the diluting segment. Three expressions have been employed for "distal delivery" of NaC1: a) V/GFR, designated as the "volume term"; b) (CNa/GFR + CH2O/GFR), the "sodium term;" and c) (CC1/GFR + CH2O/GFR), the "chloride term". The validity of these terms is discussed. Three techniques were used to increase distal delivery: 1) the administration of acetazolamide to dogs in which extracellular fluid (ECF) volume was not expanded (grop 1); 2) "moderate" volume expansion (group 2); and 3) "marked" volume expansion (group 3). CH2O/GFR increased progressively with rising values for "distal delivery" regardless of which term was used to calculate the latter. With all three delivery terms, differences in distal NaC1 reabsorption emerged between the two volume-expanded groups, though only with the "chloride" term did substantial differences also emerge between the nonexpanded group 1 dogs and both volume-expanded groups. In group 1, values for CH2O/GFR increased in close to a linear fashion up to distal delivery values equal to 24% of the volume of glomerular filtrate. However, at high rates of distal delivery the rate of rise of CH2O/GFR was less in group 2 than in group 1 and the depression of values was even greater in group 3. Within the limits of the techniques used, the data suggest that volume expansion inhibits fractional NaC1 reabsorption in the diluting segment of the nephron in a dose-related fashion. The "chloride" term was found to be superior to the "volume" and "sodium" terms in revealing these changes.