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A 16-year-old boy with type I GSD has been treated with parenteral nutrition, continuous intragastric feeding, and portacaval shunt. The effects of these treatment methods have been compared with reference to changes in blood chemical values, platelet function, and clinical status. Each of the treatment procedures was effective in improving clinical status and in reducing the levels of blood lipids. Portacaval shunt was less effective than the other treatment methods in reducing either the uric acid levels or the tendency to develop hypoglycemia, or in correcting the platelet dysfunction. Despite these observations the patient has grown 4 inches in 5 months since introduction of the shunt. The implications of these findings are discussed.

Temporal bone findings in a 75-year-old man with a 34-year history of unilateral Meniere syndrome showed severe subepithelial fibrosis of the intermediate endolymphatic sac, bilaterally. The nonhydropic side showed narrow patency of the utriculoendolymphatic valve together with questionable "fullness" of the saccule duct and slight bulging of the adjacent free wall of the saccule. The vein of the vestibular aqueduct, on the same side, drained spontaneously into a large vascular channel within bone marrow, in its course toward the posterior fossa, before joining with the tissue of the vestibular aqueduct. This was not present on the hydropic, Meniere side. The collateral drainage of the vein of the vestibular aqueduct may have acted as a spontaneous shunt, compensating the fluid mechanics to an extent where incipient hydrops, only, was present.

A distal limb of a ventriculojugular shunt became disconnected in a child treated for hydrocephalus and chest films showed the tubing had migrated into the right heart and superior vena cava. — The tubing was retrieved under fluoroscopic control by a snare technique (a doubled-over guide wire introduced through a red Kifa catheter via the femoral vein).

Clinical, radiologic, hemodynamic, and autopsy correlations were obtained from five patients with acute rupture of the interventricular septum as a complication of myocardial infarction. In such cases, confirmation of the diagnosis and accurate quantification of the wide spectrum of the resulting left-to-right shunt are prerequisites when deciding between medical treatment and emergency cardiac operation. A Swan-Ganz flow-directed catheter enabled measurement of the shunt magnitude and simultaneous exclusion of papillary muscle dysfunction or rupture quickly at the bedside, with minimal inconvenience to the patient The shunt volume correlated with the rupture size at autopsy. Serious biventricular heart failure developed without exception. As a new hemodynamic finding, a patient with excessive volume overload may show signs similar to cardiac tamponade in the atrial pressure tracings. Systolic murmur characteristics were not related to the size of the shunt, but portable chest x-ray examination provided a semiquantitative clinical means of assessing the shunt size.

A new minor surgical procedure for ascites has been devised wherein a specially designated one way pressure activated valve is implanted to create a permanent peritoneo-venous shunt. The normally closed valves opens only when the peritoneal pressure rises 3-5 cm higher than the intrathoracic venous pressure thus preventing backflow of blood and closing the valve should the venous pressure rise from the over-infusion of ascitic fluid. The procedure has been performed on 45 patients but nine were terminal at the time of surgery. Prolonged relief of ascites occurred in 28 of 37 cases.

A continuous murmur typical of patent ductus arteriosus persisting, for days or weeks was noted in 18 of 100 neonates whose birth weights were less than 2,500 grams. The murmur became apparent during the first two weeks of life in 13 infants. There was evidence of a significant left-to-right shunt in three infants, only one of whom developed heart failure. The murmur was last heard at 20 to 69 days in 16 of 17 surviving infants; in 15, it had disappeared by 3 to 29 weeks. In only one infant, now over a year of age, does it seem that the ductus is unlikely to close spontaneously.

A patient with an intrarenal arteriovenous fistula following nephrolithotomy is reported. The main clinical symptom was hematuria. The value of selective renal arteriography for diagnosis and for planning of operation is stressed. Surgical management of intrarenal arteriovenous fistulas consists of ligation of the vessels feeding and draining the shunt.

An anastomosis between the recurrent part of the right vagus nerve and the dorsal ramus of the interior laryngeal nerve was performed in dogs. After allowing 12 months for nerve regeneration, it was shown that isolated reinnervation of the cricoarytenoid muscle had occurred. The following results were shown by this work: Normal action potentials of those muscles experimentally reinnervated, and denervation potentials in the remaining laryngeal musculature. Physiologic synchrony on abduction of both vocal cords. Histochemical evidence for almost normal acetylcholinesterase activity in the reinnervated musculature, and diminished enzyme levels in the denervated endolaryngeal muscles.

We have reviewed the clinical and catheterization data and pathologic findings in ten infants under one month of age with isolated severe aortic valve stenosis. All presented with evidence of progressive cardiac failure and diminished cardiac output. A hyperdynamic right ventricular impulse was present in nine infants who were subsequently shown to have a left-to-right atrial shunt, through a stretched patent foramen ovale. Calculated aortic valve area ranged from 0.12 cm 2 /m 2 to 0.29 cm 2 /m 2 . Three patients died prior to surgical intervention because of low cardiac output and refractory metabolic acidosis. Aortic valvotomy was attempted in the remaining seven. Five died intraoperatively or in the immediate postoperative period and one other died six months after surgery. Necropsy was obtained in all nine patients who died. We believe that, in this group, the clinical course reflects fetal and postnatal hemodynamics. We have discussed the probable hemodynamic factors which occur and have related them to the morphological development of aortic stenosis in these infants. The poor prognosis of these patients probably results from a combination of factors which include left ventricular outflow obstruction, often associated with a small aortic annulus, a markedly thickened left ventricle, and left ventricular malfunction secondary to subendocardial ischemia and/or endocardial fibroelastosis.

Radioactive microspheres (15 ± 5 μm) were used to investigate capillary and shunt blood flow in the nasal mucosa of the cat. Capillary flow was about 0.5 ml/g/min. An abundant arterio-venous shunting was simultaneously recorded. Shunt flow was 57 ± 11% of total blood flow. Sympathetic stimulation resulted in a greater reduction in shunt flow than in capillary flow, indicating that increases in sympathetic nerve activity will result in a redistribution of flow from shunt to exchange vessels.

A two-year-old child was hospitalized with a diagnosis of laryngotracheo-bronchitis. His airway symptoms, however, were the presenting manifestations of superior vena caval thrombosis around a ventriculoatrial shunt. Subglottic edema obstructed his airway initially; this was relieved by tracheostomy. Later, progressive tracheobronchial compression from the dilated superior vena cava and its collateral vessels almost proved fatal. Removal of the shunt allowed blood to flow through the thrombus. This relieved the patient's severe respiratory distress within hours.

The porto-systemic collaterals were studied in rats 14 weeks after making a 1.2-mm stenosis of the portal vein. A prominent shunt from the coronary-lienal vein to the left suprarenal and renal veins w

✓Silicone tubing (Silastic) used for ventriculoperitoneal shunts induces a fibrous connective tissue sheath around the tubing in children and adults. Two children examined 8 and 3 years after subcutaneous implantation showed a complete tube of dense fibrous connective tissue around the silicone tubing. The reaction was entirely quiescent. These tubes of connective tissue were apparently capable of conveying cerebrospinal fluid for some months after the silicone tubing was disconnected from the pump or pulled out of the abdomen.

The techniques used for 4 limb angiography in 10 patients with chronic renal failure on long term hemodialysis are described.It is suggested that to show the arteries of the upper and lower limbs bilateral axillary and femoral artery cannulation should be used. In the arms follow-through to the venous phase is usually adequate to show the veins but in the legs, because of hemodilution, separate bilateral ascending phlebograms are required.

Echocardiograms were performed on premature infants with a patent ductus arteriosus (PDA) in order to assess the magnitude of the left-to-right shunt. Ten of 14 infants with a hemodynamically significant PDA required ligation because of intractable cardiac failure. Twenty infants served as controls. These consisted of seven infants without heart disease, ten infants who were examined echocardiographically after surgical ligation of a PDA, and three infants who were considered to have closed the PDA spontaneously prior to echocardiographic examination. We measured the left atrial (LA) and aortic root (Ao) diameters and an LA/Ao ratio was used to assess left atrial size. The LA/Ao ratio was 1.28 ± 0.23 ( sd ) in the group with PDA and 0.86 ± 0.10 in the control group. Infants who required surgical ligation all had an LA/Ao ratio above 1.15 (mean 1.38 ± 0.19). After ligation of the PDA the ratio returned to normal levels (0.87 ± 0.12). Sequential studies performed on seven infants demonstrated the usefulness of the LA/Ao ratio in following the changes in left-to-right ductus shunting and left ventricular failure as well as the effects of medical and surgical therapy. The LA/Ao ratio is helpful in differentiating infants with respiratory distress due to ductus shunting and left ventricular failure from intrinsic pulmonary disease.

The incidence of shunt infection after ventriculoatriostomy was reviewed in 104 patients undergoing 160 operations. A shunt infection was identified within 6 mo after operation in 20% of the 105 operations in which prophylactic antibiotics were not used, and in only 5% after 55 operations in which they were given. This suggests that the prophylactic use of antimicrobial drugs after shunt operations may have merit.

The echocardiographic patterns of increased right ventricular dimension and abnormal septal motion were observed in a patient with a large pelvic arterio-venous (A-V) shunt and no associated cardiac lesion. Although there was evidence of incomplete closure of the shunt four months after surgery, the right ventricular dimension had returned to normal but abnormal septal motion persisted. While these echocardiographic findings cannot be attributed specifically to the shunt in this patient, such a lesion should be considered in patients with these echocardiographic patterns and no cardiac abnormality.

In patients with tetralogy of Fallot, those with intrapulmonary pulmonary arterial stenosis (IPPS) diagnosed by angiocardiographic measurement and pulmonary scintiscanning seem to be poor candidates for one stage total correction. The results obtained in the present study indicate the significance of developmental state of pulmonary vascular bed in establishing indication for total correction of tetralogy of Fallot. In view of the improvement of IPPS following the shunt operation, second-stage total correction after the Blalock shunt is recommended in those with IPPS.

Juhl, E., Bruusgaard, A., Hippe, E., Korner, B., Quaade, F. & Baden, H. Vitamin B12 depletion in obese patients treated with jejunoileal shunt. Scand. J. Gastroent. 1974, 9, 543–547.In 10 obese pat...

A 15 year old Indian boy was discovered to have a very loud continuous murmur over the right supraclavicular region. Cardiac catheterization findings demonstrated an atrial septal defect and a left to right shunt at the level of the right innominate vein. At thoracotomy, total anomalous pulmonary venous drainage was found. The dilated vertical vein arising from the common pulmonary vein was narrowed to about 1.2 cm in diameter at its site of entry into the right innominate vein, causing blood to flow into the latter vessel in a jet-like fashion, thus producing the loud continuous murmur.

When a fulminant case of superior vena caval obstruction is diagnosed and the distressing symptoms are prominent, a shunt operation with a composite vein graft from innominate vein to right atrium may completely alleviate the symptoms. This has been shown in the case of a 53-year-old man, whose superior vena caval obstruction was due to a bronchogenic carcinoma, with complete relief of the obstructive symptoms.

Nineteen patients were studied angiographically following transcranial temporal artery—middle cerebral artery anastomosis surgery. All anastomoses remained patent up to 27 months. Flow patterns varied from filling of the entire middle cerebral triangle via the anastomosis to filling of one or two branches. Clinical improvement varied inversely with the degree of middle cerebral artery filling via the shunt. Best results occurred in patients having repeated transient ischemic attacks without a fixed neurologic deficit. On a second postoperative study 2 of 5 patients showed increased flow via the anastomosis. This procedure may be prophylactic in preventing massive cerebral infarction in progressive arterial disease.

A case with an extraordinary arteriovenous shunt and a systemicpulmonary arteriovenous shunt has been reported. The etiology may be previous pulmonary tuberculosis and pleuritis treated with artificial pneumothorax.

An unusual case of tetralogy of Fallot, in which a long-present side-to-end shunt between the descending thoracic aorta and the left pulmonary artery had to be closed as the first part of a corrective cardiac procedure, has been presented. The pulmonary artery was separated from the aorta and a graft was attached to it through a posterolateral incision. Then the mediastinotomy incision was made for open repair of the congenital cardiac anomaly and for attachment of the graft to the pulmonary trunk. The marked cyanosis which occurred immediately upon closing the shunt prompts the suggestion that, in circumstances such as this, the surgeon should be prepared immediately to place the patient on partial cardiopulmonary bypass through previously inserted femoral or iliac catheters. This general plan may offer the safest approach in taking down functioning Potts anastomoses as the first step in the complete repair of tetralogy of Fallot in other situations in which the aorto-pulmonary anastomosis can be most safely and effectively dealt with through a posterolateral incision.

A total of 101 infants and children with cyanotic congenital heart disease and inadequate pulmonary blood flow underwent an ascending aorta-to-pulmonary artery anastomosis at the University of Florida from January, 1964, to December, 1972. The operative mortality was 37%. The 64 surviving patients have experienced improved oxygenation of the blood and disappearance of hypoxic episodes for up to 8 years postoperatively. Two of 4 late deaths were related to inadequate pulmonary blood flow. Cardiac catheterization was performed in 16 patients 1 week to 6 years after the operation. Eight patients later underwent complete cardiac repair and discontinuance of the shunt.

Presentation of Case A 31-year-old woman was admitted to the hospital because of fever. At the age of 10 years she entered another hospital because of hematemesis. The physical examination disclosed splenomegaly and massive hepatic enlargement, and an upper gastrointestinal series revealed esophageal varices. A splenectomy, a splenorenal shunt and a liver biopsy were performed; microscopical examination disclosed congestive splenomegaly and congenital cystic disease of the liver. Nine years later she was admitted to a second hospital because of fever and was found to have septicemia due to Escherichia coli, with multiple septic emboli to the lower lobe of . . .

A series of 24 patients with cirrhosis have undergone mesentericocaval shunt operations for the relief of portal hypertension. Overall the results have been satisfactory. Four of the five patients treated as an emergency and 17 of the 19 who had the operation two to six weeks after haemorrhage had been controlled left hospital alive and well. Separation of the patients into three categories according to the findings of clinical and biochemical tests, however, showed that subsequent survival was satisfactory for patients in categories A and B but that all four patients in category C had died within one year after surgery. Assessment at three months showed that in three patients moderate hepatic encephalopathy had developed.Evidence that the shunt remains patent was shown by a low incidence of repeated gastrointestinal haemorrhage and a marked diminution in variceal size in 18 of the 19 cases examined serially. Radiographic techniques for confirming shunt patency were compared and cannulation of the graft via the femoral vein was found to provide a reliable and rapid means of assessment.

In this malformation the difficulty in sending blood through the stenosed pulmonary valve leads to right heart enlargement and to functional patency of the foramen ovale, through which a venousarterial shunt occurs. The onset of symptoms and their severity depend on the size of the opening in the pulmonary valve. Characteristic physical findings and results of fluoroscopy, electrocardiogram, circulation time, arterial blood studies, exercise test, angiocardiogram, and cardiac catheterization are presented. Diagnosis is important because these patients develop cardiac failure following at Blalock-Taussig operation, but may be greatly helped by pulmonic valvulotomy.

The case of a 55-year-old man with an apical coronary artery aneurysm is presented. The aneurysm was in communication with the dilated descending branch of the left coronary artery. There was no shunt. The physical findings suggested aneurysm with a fistula to the left ventricle. Coronary angiography demonstrated a localized aneurysm in the wall of the left ventricle, but no fistula. The aneurysm was surgically obliterated and the patient made a good recovery. This seems to be the first described case of coronary artery aneurysm without fistula to be fully diagnosed prior to surgical exploration.

This review and supporting experiments in which the left pulmonary artery was ligated on the first day of life, have clearly implicated an increased pulmonary blood flow from the time of birth as an important factor in the genesis of pulmonary hypertension. Ninety-eight cases of unilateral absence of a pulmonary artery, including four new cases, have been collected from the world's literature and are reviewed in this paper. This lesion is frequently undiagnosed and may be more common than is generally appreciated as shown by the marked increase in case reports in the past ten years. The blood supply to the affected lung has been explained on an embryologie basis, and two distinct embryologic faults have been implicated. The incidence of pulmonary hypertension in the isolated cases was 19 per cent, and when combined with a cardiovascular shunt the incidence was 88 per cent. In view of the high incidence of pulmonary hypertension when UAPA is associated with a cardiovascular shunt, it is important to recognize these patients at an early age and correct the associated shunt to prevent or reverse pulmonary hypertension, if possible. It is evident that this lesion must be considered in the differential diagnosis of pulmonary hypertension and cyanotic congenital heart disease.

F OR many years quadrupIe ligation and excision has been the method of surgical treatment of acquired arteriovenous corn munication. Recent investigations,9 particularly in centers caring for war casualties, suggest a reduced incidence of arteria1 insuffrciency when arterial continuity is re-established by end-to-end anastomosis, homoIogous artery graft or autogenous vein graft. Figure I illustrates a situation found frequently in our series. Stimulated by these recent innovations in vascuIar surgery, we have undertaken a study of thirty-two patients afFEcted with traumatic arteriovenous IistuIa of a limb; the treatment in each had consisted of interruption of the arterial and venous circuIation in the limb by means of Iigation and excision of the involved vesseIs. SurgicaI specimens were submitted to gross and microscopic examination; questionnaires sent to patients afforded the foIIow-up evaluation of each limb treated by Iigation and excision. Particular emphasis was pIaced on adequacy of the interrupted arteria1 and venous circuIation. Although much has been written from earIy times about the arteriovenous fistula, it remained for WiIIiam Hunter in 1739 to distinguish it from a simpIe aneurysm; he was the First to give a detailed description of the signs, symptoms and physica findings of the affliction. The major cIinica1 manifestations are a harsh thrill and a bruit in the involved area; these are continuous throughout the cardiac cycie with systolic accentuation. The rapid passage of arteria1 bIood into the venous circulation may resuh in marked elevation of the venous pressure and thence in chronic venous insuffrciency with varices, edema and stasis pigmentation; aIso, one sees ulceration of the limb. Figure 2 shows the increased venous pattern and generalized enIargement of the limb with a Iong-standing fistuIa.‘*Another frequent Iinding is cardiac enlargement. One may make a diagnosis of acquired arteriovenous fistula when a thril1 and a bruit are observed in the region of a penetrating wound: the presence of chronic venous insuffrciency and increased warmth in such a limb in the region of the fistuIa is further evidence of an arteriovenous shunt. In our experience arteriography was not necessary to enable one to estabIish the diagnosis.

We have described a case of multiple and complex malformations of the coronary arterial system, i.e., single coronary artery which gave off several branches, one of which connected abnormally with the right ventricular cavity so as to produce a shunt. In addition, there was severe pulmonary stenosis with a high right ventricular pressure. This, in turn, maintained a right-to-left shunt through the abnormal coronary vessel. Another, similar shunt was present at the atrial level through an atrial septal defect. These malformations were suspected clinically. Specialized studies (catheterization and angiocardiography) confirmed the diagnosis. The patient was operated upon for ligation of the abnormal coronary vessel and correction of the pulmonary stenosis. He died as a result of cardiac arrest. The hemodynamic pattern of this unusual case is discussed.

Two cases of patent ductus arteriosus with intermittent reversal of the shunt are described. The bidirectional flow was demonstrated during life by cardiac catheterization, simultaneous right brachial artery and femoral artery blood oxygen analyses, oximetric and dye dilution studies, and by angiocardiography. The clinical picture and laboratory findings to be expected in cases of ductus arteriosus with intermittent reversal of the shunt are presented and the extremely high operative mortality in such cases is stressed. Finally, the possible pathogenesis of the marked pulmonary hypertension is discussed briefly.

SUMMARY A technique is described for producing chronic hypoxia in calves by partial diversion of the inferior vena cava into the left atrium. The arterial hypoxia produced by an intracardiac shunt did not produce pulmonary hypertension, though a comparable degree of hypoxia produced by breathing 10 to 15 per cent oxygen did produce pulmonary hypertension. This difference in response suggests that the receptor mechanism initiating pulmonary hypertension is more sensitive to oxygen concentration in alveolar gas than in pulmonary capillary blood. The technique of cardiopulmonary bypass in young calves is described, as the calf may be a suitable experimental animal for several experimental cardiac procedures because of the large size of the structures present. The technical assistance of Dr. C. E. Eastin, D.V.M., was very helpful during these experiments.

SummaryAn examination of hexosemonophosphate shunt dehydrogenase activity in the rat exudate polymorphonuclear leukocyte reveals an exceedingly high NADPH generating capacity which is not modified by changes in composition of diet or alterations in thyroid status of the animal. This is in marked contrast to the hepatic shunt dehydrogenases which show marked alterations in response to the above stimuli. Thus, leukocyte HMPS dehydrogenase activity cannot be used as a peripheral index of hepatic shunt dehydrogenase activity.

Summary The term compensated patent ductus arteriosus is suggested for the condition of those patients in whom the arteriovenous shunt is small and there is no appreciable effect on the peripheral circulation or cardiac reserve. Such cases may be reliably diagnosed by the characteristic murmur in the absence of any other sign or symptom. The murmur, as illustrated by the phonocardiogram, is crescendo in systole, continues into diastole, becoming decrescendo. The term uncompensated patent ductus arteriosus may be used forthose patients having a large arteriovenous shunt with evidence of present or impending circulatory insufficiency. In such cases, in addition to the characteristic murmur, there is a low diastolic pressure with the peripheral signs of aortic regurgitation, cardiac enlargement, pulmonary congestion, and impaired growth and development of the child. These cases may be compared to “free” aortic regurgitation. Surgical ligation is indicated for the uncompensated patent ductus arteriosus at whatever age signs of circulatory embarrassment appear. For patients with a compensated ductus, a policy of watchful waiting is advised.

SUMMARY A method of evaluating preservation techniques by means of extracorporeal perfusion on an arteriovenous shunt is presented.

Trisomy 17/18 is accompanied by multiple congenital anomalies that usually cause death in infancy. The radiological picture may be distinctive. The most constant features are: slender, slightly irregular ribs and clavicles; congenital heart disease with a left-to-right shunt; short great toes, non-ossified middle phalanges of the second or third to fifth toes and small, triangular terminal phalanges. The radiological features of Trisomy 13/15 are also described; the two syndromes resemble one another but the X-ray picture is less complete in the latter.

An arteriovenous shunt with a side arm was used for the intravenous feeding of a patient who had bowel resected from the third part of the duodenum to the ascending colon and who could no longer be maintained by indwelling venous catheters. The nutrient solution is prepared in plastic bags by a procedure that minimizes contamination and permits preparation and administration at home. Infusion is given in a period of nine hours or less, a special pump, filter and valve being employed. The patient has been maintained in good nutritional status by this means for a period of many months.

Infants with severe respiratory distress were investigated by analysis of gases in arterial and venous blood during the breathing of pure oxygen. The results indicated that the infants suffered from a reduced alveolar ventilation and from right-to-left shunt. Possible mechanisms for these disorders are discussed.

The vascular images were studied in 37 cases of primary liver cancer.In well differentiated hepatoma, vascular proliferation, tumor stain and A-V shunt formation are regarded as specific features.In anaplastic hepatoma and cholangiocarcinoma, however, it is difficult to assign a definite pattern, apart from the peculiarity of tumor vessels, relating to the histologic diagnosis of the tumor.Formation of collateral circulation in cholangiocarcinoma is considered to be a relatively rare finding.

1. 1. Three operative techniques to produce chronic cyanosis in dogs are described and the results compared. 2. 2. There were no significant differences among the survival rates with these techniques. 3. 3. Partial diversion of inferior vena cava to left atrium produced chronic cyanosis in only 38% of the surviving animals. 4. 4. Following direct anastomosis of inferior vena cava to left atrium, 71% of survivors were cyanotic but secondary stenosis of the shunt occurred in the remaining 29%. 5. 5. With the graphite-benzalkonium-heparin treated Dacron grafts between the inferior vena cava and the left atrium, all surviving dogs were deeply cyanotic and secondary stenosis of the graft was not found at autopsy. This technique seems to be the method of choice. 6. 6. The importance of prolonged intensive chemotherapy is emphasized because bacterial endocarditis was the main cause of late death in the period followed. 7. 7. A mild metabolic acidosis with compensatory respiratory alkalosis was found in the cyanosed dogs.

Growth study of 25 acyanotic children with left-to-right shunt and 5 cyanotic children with right-to-left shunt (tetralogy of Fallot) is reported. Data were collected at the time of operation and on followup 4 1/2–12 months later. No significant differences in the two measurements were seen. Weight was more impaired at the time of operation than height. The literature is reviewed.