Perioperative management of pulmonary hypertension after heart transplantation in childhood.

BACKGROUND: Pulmonary hypertension is responsible for a substantial part of perioperative and postoperative mortality and morbidity after cardiac transplantation. Treatment of right ventricular failure after increased pulmonary vascular resistance is difficult especially in infants and children. Therefore we started a preventive therapy of pulmonary hypertension after cardiac transplantation to avoid right ventricular failure and compared the results with a group of patients with conventional therapy. METHODS: Group 1 (n = 13), with transplantation from 1988 to 1991, was treated with vasodilators when symptoms of right ventricular failure developed. Group 2 (n = 19) had preventive treatment with prostaglandin E1 (PGE1), the phosphodiesterase-III inhibitor enoximone, and alkalinazation starting during weaning from cardiopulmonary bypass. RESULTS: Six patients in group 1 died; four of them as the result of right ventricular failure in the immediate postoperative course despite aggressive treatment. In group 2 there were three deaths as the results of rejection (2) and infection (1). None of these patients developed right ventricular failure (p = 0.02). Cold ischemic time, extracorporeal circulation time, and waiting time before transplantation were significantly longer in group 2. Side effects of this preventive therapy were not observed. CONCLUSIONS: We conclude that prophylactic therapy of pulmonary hypertension with vasodilators in infants and children after heart transplantation is safe and effective in preventing right ventricular failure in the postoperative course.

Diverticulum of the right ventricle associated with ventricular septal defect.

A true diverticulum of the ventricle is a very rare congenital cardiac lesion which is characterized by a normal myocardial wall and active participation in the ventricular contractions. Diverticula of the right ventricle may originate from the apical or anterosuperior wall. We report three patients with an anterosuperior diverticulum of the right ventricle associated with a perimembranous ventricular septal defect, one of whom additionally had obstruction of the right ventricular outflow tract. These patients presented clinically with symptoms related only to their associated defects. The right ventricular diverticulum was diagnosed by angiography in all, but was also seen on cross-sectional echocardiography in one patient. The ventricular septal defect was repaired through an incision of the diverticulum, which was reduced in size at the same time. Complete resection appears necessary only if severe endocardial fibrosis is present within the diverticulum, because such a diverticulum does not contribute to right ventricular contraction, but rather is at risk of rupture.

Clinical experience with heart transplantation in infants.

OBJECTIVE: Orthotopic heart transplantation has become an accepted therapeutic concept for adult patients with endstage heart disease. In newborns and infants this procedure is still a matter of discussion because of unknown long-term results and the lack of donor organs. METHODS: Since March 1988 we have performed 40 orthotopic heart transplantation in 39 infants who were from 1 to 280 days of age. Indications for transplantation included hypoplastic left-heart syndrome (n = 28), dilative cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and other complex structural anomalies (n = 3). The mean waiting period for transplantation was 53 days. A donor-recipient weight ratio up to 4.0 was accepted. Profound hypothermic circulatory arrest was used for graft implantation in all those patients who required extensive aortic arch reconstruction (71%). The initial immunomodulation was based on Cyclosporine, Azathioprine and Prednisolone. Patients who underwent transplantation during the first 6 weeks of life received a chronic single-drug therapy with Cyclosporine after 1 year. RESULTS: There were six peri-operative deaths caused by drug-resistant right-heart failure in three cases, humoral rejection (n = 1), CMV infection (n = 1) and multi organ failure (n = 1). One infant died late, due to rejection. The actuarial survival rate for the entire group is now 82%. There is a remarkable influence of increasing experience. Whereas six of 15 infants who had heart transplantation between 1988 and 1993 died early post-operatively (survival rate: 60%), only one late death occurred among 24 recipients in the period from 1994 to April 1997 (survival rate: 96%). Episodes of rejection occurred once or several times in about half of the patients in this series (48%). All surviving children are living at home in excellent condition. CONCLUSIONS: Heart transplantation during early infancy is a rational and durable therapy for heart diseases with irreversible myocardial failure or severe structural anomalies. The intermediate-term results have been encouraging in many centers, but more data must be accumulated to determine the sequelae of chronic immunosuppression. The lack of donor organs remains one of the major problems in pediatric heart transplantation.

Surgical therapy of pulmonary thrombosis due to candidiasis in a premature infant.

We report a case of surgical removal of a pulmonary thrombosis in a premature infant with candidemia. The pulmonary thrombotic material contained candida albicans. Following surgical therapy the child recovered soon and candidemia did not recur. We conclude that surgical treatment of pulmonary thrombosis can be successfully done even in premature infants.

Myxoma of the aortic valve in a child.

Myxomas are predominantly located in the right on left atrium. We report an unusual case of a myxoma adherent to the aortic valve. The child presented with symptoms of subaortic stenosis. By invasive methods a tumor was diagnosed located at the aortic valve. After surgical removal the child recovered soon and is doing well. According to our knowledge this is the first report of a myxoma of the aortic valve.

Cardiac transplantation in a neonate with endocardial fibroelastosis.

An investigational orthotopic cardiac transplantation was performed to manage subendocardial fibroelastosis in a neonate. No unmanageable technical complications arose from the transplantation. Postoperative observation showed the infant developed normally except for moderate cerebral palsy.

Fenestration in extracardiac conduits in children after modified Fontan operation by implantation of stent grafts.

Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surgical fenestrations in two children and absent fenestration in one. As an emergency procedure, in all patients the conduit was perforated by transcatheter intervention in order to create a connection to the anatomical right atrium. Following balloon dilatation of the perforated conduit, in all three patients covered stent grafts were placed in the newly created defect to attain a reliable communication. Patency of the fenestration was demonstrated by angiogram and any leakage was ruled out. Cardiac output improved and severe pleural effusion and ascites subsided. Right-to-left shunt could be demonstrated by echocardiography at follow-up after 7 months (median) in all three patients. Oxygen saturation remained stable between 85 and 90%. These preliminary results suggest that stent graft fenestration can serve as a valuable tool in failing Fontan circulation, particularly in patients with an extracardiac conduit. Covered stents have the potential to reduce the acute risk of bleeding and they help to prevent early spontaneous closure of the newly created fenestration.

[The determination of heart minute volumes and systolic time intervals using echo- and Doppler echocardiography in severely ill premature infants]. / Zur Bestimmung von Herzminutenvolumina und systolischen Zeitintervallen mittels Echo- und Dopplerechokardiographie bei schwerkranken Frühgeborenen.

The here represented echo- and doppler-echocardiographic measurements of critically ill preterm infants demonstrate the possibility to watch closely to hemodynamic changes due to new treatments of these patients, thus to gather more information than with the usual monitoring of a neonatal intensive care unit. Using systolic time intervals and cardiac output, measured in the ascending aorta and the pulmonary truncus by echo- and doppler echocardiography, gives more ease and accuracy in actual therapeutic decisions. This is demonstrated with the use of dopamine, prostaglandin E1 and indomethacin.

[Pulmonary insufficiency in childhood. Contribution to diastolic murmurs (author's transl)]. / Pulmonalinsuffizienz im Kindesalter. Ein Beitrag zur Frage des diastolischen Geräusches.

Diastolic murmurs in childhood are in most cases due to pulmonary insufficiency. They may be functional (Graham-Steel-murmurs, for instance with idiopathic pulmonary ectasis or pulmonary hypertension), but are more and more the symptom of postoperative pulmonary insufficiency. Diastolic murmurs of pulmonary insufficiency were found after operations of valvular and infundibular pulmonary stenosis in 92,5% of the cases (40 children) and after total correction of tetralogy of Fallot in 89% of 27 children. The diastolic murmur of pulmonary insufficiency is very good recorded in phonocardiography; with postoperative heart catheterisations and angiocardiography there is good estimation of the degree of the pulmonary insufficiency. There is no doubt about the curative effort of operations in these congenital heart defects, and postoperative pulmonary insufficiency does not impair the operative improvement in childhood. Till now long term observations of these cases up to late adulthood cannot be possible; regular pediatric cardiologic examinations of these operated children are mandatory.

[Primary pulmonary hypertension and abnormalities of pulmonary arteries]. / Primäre pulmonale Hypertension und Anomalien der Lungenarterien

Case report of a 5 year old boy with a well diagnosed primary pulmonary hypertension, combined with anomalies of the pulmonary arteries of the left upper lobe of the lungs with angiographically loopy appearance. Congenital heart and vascular disease did not exist; AV-fistulae were not found. There is no proof of a connection between the anomalies of the pulmonary arteries and pulmonary hypertension. There was no similar case in the current literature to be found.

The horizontal interventricular septum. Three cases with different ventriculoarterial connections.

Three hearts sharing the anatomical peculiarity of a horizontal interventricular septum are described. In one, both great vessels originate from the superior right ventricle, in the other the great arteries are connected normally, the third one shows a single aortic trunc. It is concluded that the horizontal interventricular septum has to be considered as an independent cardiac deformity occurring with most types of ventriculoarterial connections. Assuming rotation of the bulboventricular loop around a base-apex-axis as the basic ontogenetic event one can trace this morphology back to its original layout. It is, therefore, possible to classify each case according to the usual nomenclature regarding the horizontal septum and the upstairs-downstairs arrangement of the ventricles as an addition. This embryological event of rotation is considered the product of a restraightening process of the previously looped cardiac tube.

[Cervical aortic arch--symptoms and diagnosis in 2 children]. / Zervikaler Aortenbogen--Symptome und Diagnostik bei zwei Kindern.

Two children with cervical aortic arch are presented. This anomaly is very rare. In one patient cervical aortic arch Type A (Haughton) was associated with a ventricular septal defect and patent ductus arteriosus. In the other child we report the only case of cervical aortic arch (Type B) in association with partial anomalous pulmonary venous return.

[Obliterating changes of pulmonary veins as the cause of pulmonary hypertension]. / Obliterierende Lungenvenenänderungen als Ursache eines pulmonalen Hochdruckes

The clinical course and the necropsy study in a 9,7 year old boy with primary vascular pulmonary hypertension showed that it was due to an intrapulmonary venoocclusive disease. The inter- and intralobular veins and the venoles revealed fibrous intimal thickening up to subtotal obstruction or total occlusion. The etiology remains unknown. There were no thrombi of veins or arteries within the lung. It is supposed the intimal proliferation due to an unknown etiologic agent is primary and thrombosis secondary.

[Modern differentiation possibilities of angiocardiography in childhood]. / Moderne Differenzierungsmöglichkeiten bei der Angiokardiographie im Kindesalter

1. Angiocardiography plays an irreplaceable method of investigation in the diagnosis of cardiac diseases in infancy and childhood. In many cases it is the "crown" of the diagnosis. 2. Cine-angiocardiography is indispensable in paediatric cardiology. In infancy, in rapid heart rates and in complicated malformations of the heart, only a movie film (35 mm) can register completely all the anatomic and haemodynamic details. 3. In certain cases the serial angiocardiography is also necessary in childhood. Today two methods of radiography can be made use of: the direct recording by means of rapid film changers on full size X-ray films and the indirect method on 70- or 100-mm size films. 4. According to our experience, the serial angiocardiography recordings on 70-mm size films have more advantages than the usual full size films (35 x 35 cm). In the interpretation of the 70-mm-size films there is no loss of information, and the radiators dose is 1/10 lower than in AOT-size.

[Sonographic image of intrarenal and intra-arterial fungus thrombi in a newborn infant with Candida infection]. / Sonographische Darstellung von intrarenalen und intraarteriellen Pilzthromben bei einem Frühgeborenen mit Candida-Sepsis.

Shortly after birth a preterm infant suffering from aspiration syndrome and subsequent Pseudomonas aeruginosa sepsis showed signs of renal insufficiency and mycotic infection: Yeast cells were identified in several urinalyses; there was also an increasing anti-Candida IgM antibody titer. At the same time sonographic examinations revealed an increasing echogenicity of the renal cortex and echogenic masses of variable size which did not cause acoustic shadows in both enlarged kidneys. A few days later, we found a right-sided hydronephrosis caused by an intraureteric prevesical mass of equal echogenicity. As we could observe sonographically, the aggressive antimycotic therapy was successful. Eleven weeks later there were signs of cardiac insufficiency. An angiographically demonstrated filling defect within the pulmonary artery showed the same sonographic findings as the previously found intrarenal masses. The baby underwent embolectomy and recovered. The thrombotic material contained yeast cells giving evidence of systemic Candidiasis. Provided appropriate equipment is available, ultrasound today is an excellent non-invasive screening and follow-up method not only for echoencephalography, but also for more complicated neonatal problems as seen here. The detailed observation of a changing echogenicity of the renal cortex and pelvis is important and often allows a decisive diagnostic clue before other radiological methods become conclusive.

Severe digoxin intoxication in a child treated by infusion of digoxin-specific Fab-antibody-fragments.

Accidental digitalis ingestion in children is a rare, but potentially life-threatening emergency. We report the case of a 2 10/12-year-old boy with accidental ingestion of 6 mg beta-Acetyl-digoxin. Soon after admission, the boy developed sinus bradycardia, SA and AV-block, of increasing severity without circulatory impairment. As the serum digoxin level reached 21.7 ng/ml digoxin-specific Fab-antibody-fragments were used to bind free serum digoxin. Immediately after infusion, serum free digoxin was below the detection limit, whereas total digoxin peaked at 219 ng/ml 5 h thereafter. The arrhythmias did not subside totally, so that in addition, a transvenous pacemaker was placed, but never used. The antibody-infusion was well tolerated and the boy was discharged in good health.