Proposal for a new definition of congenital complete atrioventricular block.

The classic old definition of congenital heart block by Yater (1929) is still generally accepted: 'Heart block established in a young patient. There must be some evidence of the existence of the slow pulse at a fairly early age and absence of a history of any infection which might cause the condition after birth: notably diphtheria, rheumatic fever, chorea and congenital syphilis'. However, other definitions are used. We systematically reviewed 1825 cases from 38 separate studies. We conclude that complete AV blocks detected in utero in the absence of structural abnormalities differ from blocks detected later in life with respect to pathogenesis (they are generally associated with maternal anti-Ro/SSA antibodies), poorer childhood prognosis, increased risk of developing late-onset dilated cardiomyopathy, different maternal clinical features and increased risk of recurrence in future pregnancies. For these reasons we propose a new modern definition of congenital complete AV block which might be acceptable to cardiologists, rheumatologists, pediatricians and obstetricians: 'an AV block is defined as congenital if it is diagnosed in utero, at birth or within the neonatal period (0-27 days after birth)'.

Pregnancy in congenital cardiac disease: an increasing challenge for cardiologists and obstetricians -- a prospective multicenter study.

OBJECTIVES: Aim of this study was to assess the occurrence of pregnancy-related complications of mother and child during pregnancy, delivery and puerperium in women with CCD prospectively. STUDY DESIGN, POPULATION: This prospective multicenter study included 122 pregnancies in 106 women with CCD (72 with, 34 without previous cardiac surgery). Patient age was 17-44, median 26 years. Cardiac and non-cardiac complications, mode of delivery, abortion, and CCD of the newborn were assessed. RESULTS: Initially all women were in Functional Class I or II. Worsening during pregnancy occurred in 25.5% (n=27), mainly during the second and third trimester. Significant problems due to bleeding, hypertension, rhythm disturbances, endocarditis, liver congestion, increasing cyanosis or death, occurred in 11.3%. Twelve per cent of deliveries were premature. Five women had therapeutic abortion, nine spontaneous abortions, nine preterm births, and one intrauterine death. Seventy-nine per cent (n=85) delivered spontaneously; 21.3% (n=23) had caesarean section. Of the 111 live born children, 5.4% (n=6) had a CCD. CONCLUSIONS: Most women with CCD and a good functional class before pregnancy tolerate pregnancy without major problems. However, pregnancy may induce serious cardiac and obstetric complications. The specific risks require an individualized multidisciplinary patient-management by experienced physicians.

[Interventional occlusion of interatrial communication after modified Fontan operation]. / Interventioneller Verschluss von interatrialen Verbindungen nach modifizierter Fontanoperation.

UNLABELLED: After modified Fontan operations various communications between the systemic and pulmonary venous returns may cause persistent or increasing postoperative cyanosis. Interventional closure of these right-to-left shunts may be necessary to eliminate hypoxemia and to reduce the risk of paradoxical embolic complications. PATIENTS AND METHODS: Eighteen patients with a mean age of 5.6 +/- 4.1 (2.5-17.5) years underwent interventional closure of a right-to-left shunt 17.4 +/- 15.8 (3-60) months after a modified Fontan operation. After test balloon occlusion fenestrations were closed in 13 patients using an Amplatzer Septal occluder (n = 7), a Rashkind PDA occluder (n = 3), a CardioSeal umbrella (n = 1) and detachable coils (n = 2). Residual leaks at the suture lines between the interatrial patch and the right atrial wall were closed using detachable coils and a Rashkind PDA occluder in 2 and 1 patients, respectively. In 3 patients intracardiac venous collateral channels were closed by means of detachable coils. RESULTS: The mean aortic oxygen saturation increased from 85 +/- 4.5 (70-89)% to 91.4 +/- 2.8 (83-95)% (p < 0.001) breathing room air and the mean tunnel pressure rose from 10.7 +/- 1.8 (6-14) mmHg to 12.1 +/- 2.4 (6-16) mmHg (p < 0.001). Calculated Qs decreased from 5.15 +/- 2.1 (2.1-11.3) l/min/m2 to 3.6 +/- 1.0 (1.8-5.6) l/min/m2 (p < 0.001). Mixed venous saturation (66.4 +/- 7.4% vs 65 +/- 7%) and mean systemic arterial pressure (73 +/- 8 mmHg vs 73 +/- 9 mmHg) remained unchanged. In one patient an additional leak of the tunnel could not be closed because of an increase to more than 18 mmHg of the mean pressure in the lateral tunnel during balloon test occlusion. In 2 patients residual leaks after umbrella and coil occlusion of a fenestration and an additional venous collateral channel were closed by means of coils after 16 and 21 months, respectively. At a follow-up of 42 +/- 23 (7-99) months, mean oxygen saturation measured by pulse oxymetry was 93 +/- 2 (90-97)%. In 2 patients color-coded Doppler echocardiography revealed a minimal residual right-to-left shunt. In 2 patients contrast echocardiography demonstrated the additional presence of intrapulmonary fistulas. All patients remained free from device migration, thromboembolic events and hemolysis. CONCLUSION: After modified Fontan operations various right-to-left shunts between the systemic and pulmonary venous returns can be successfully closed using umbrella devices or coils to eliminate cyanosis and to reduce the risk of paradoxical embolism.

Catheter closure of the persistent foramen ovale: mid-term results in 162 patients.

Four different types of occluder systems were used to close a persistent foramen ovale (PFO) in 162 patients with paradoxical embolic events. Ninety-eight patients had ischemic stroke, 60 transient ischemic attacks (TIA) or prolonged reversible ischemic neurological deficit (PRIND), and 4 had peripheral arterial embolism. The age of the patients was 40.2 +/- 11.9 years and the ischemic event had happened 7 +/- 10 months before device closure. CardioSeal and Amplatzer occluders were the most commonly used devices (73 and 77 cases, respectively). Implantations were successful in all patients. Serious catheter-related complications included two device embolizations and two venous bleedings. Six patients had documented supraventricular arrhythmias within the first month after implantation, which disappeared spontaneously within some weeks without therapy in three patients; the other three patients with atrial fibrillation needed conversion to sinus rhythm. Residual leaks were found in 5 out of 116 patients who had been followed by transesophageal echocardiography (TEE) and one leak was closed by a second device. During a follow-up period of 19.4 +/- 16.2 months per patient, TIA and PRIND occurred in 3 of 116 patients. Interventional closure of PFO is a simple, effective and quick method that is superior to surgery and avoids the problems of life-long anticoagulation.

Transcatheter coil occlusion of residual interatrial communications after Fontan procedure.

OBJECTIVE: To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. DESIGN: Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. SETTING: Tertiary paediatric cardiac referral centre. PATIENTS: Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. INTERVENTIONS: Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. RESULTS: A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range 86-89%) to 92 (1.3)% (range, 89-93%; p < 0.001) and the mean (SD) right atrial pressure rose from 9.7 (2) mm Hg (range, 6-11) to 10.6 (2.4) mm Hg (range, 6-13; p < 0.05) after coil implantation. In five patients, complete obliteration of the interatrial shunt was shown by angiography after coil implantation. At a mean (SD) follow up of 10 (4) months (range, 3-15) a residual interatrial shunt was detected by Doppler colour echocardiography in only one patient, and oxygen saturations ranged from 90% to 95% (mean, 92%). There were no late coil embolisations, thromboembolic events, or haemolysis in any patient. CONCLUSIONS: Detachable coils can be used successfully to occlude residual interatrial communications after the Fontan procedure. In selected cases, in whom intended transcatheter umbrella occlusion of residual interatrial leaks is not possible, the use of detachable coils might offer a safe alternative method to eliminate interatrial right to left shunting after the Fontan procedure.

[Closure of the persistent ductus with detachable coils]. / Verschluss des persistierenden Duktus mit ablösbaren Coils.

34 patients aged 3 months to 20 years underwent transcatheter-occlusion of their patent ductus arteriosus with detachable coils (Cook). Eight patients had a residual ductus after previous implantation of a Rashkind-occluder; 7 patients had various other cardiac malformations in addition. Only 6 patients had a large ductus with a diameter between 3 and 4 mm; all had systolic-diastolic murmurs. All other patients had ductus-diameters below 3 mm; three of them had systolic-diastolic murmurs, 17 had systolic murmurs, and 8 patients had no murmur at all. The ductus was closed in 24 patients using arterial access only, in 6 patients via a venous, and in 4 patients both via venous and arterial catheterization. One coil was used in 23 patients, 2 coils in 9, and 3 coils in 2 patients. There were no complications of the intervention. Within 24 hours 31 patients (93%) had complete closure of the ductus and 32 patients (94%) after 6 months. Coil-embolisation of the persistent ductus is a quick, safe and cheap method to close a ductus and has clear advantages compared to an operation.

[Accessory mitral valve tissue as a rare cause of subaortic stenosis and valvular aortic insufficiency]. / Akzessorisches Mitralklappengewebe als seltene Ursache einer Subaortenstenose und valvulären Aorteninsuffizienz.

An unusual case of subaortic stenosis and aortic regurgitation caused by accessory mitral valve tissue in a 10 year old boy is reported. Two-dimensional and Doppler echocardiography revealed the characteristic feature of a mobile, parachute-like mass in the left ventricular outflow tract pro-lapsing into the aortic valve during systole and, thus, producing a systolic pressure gradient of 70 mm Hg between the left ventricle and aorta and causing mild aortic regurgitation. The accessory valve tissue was completely excised via an aortotomy without injury to the normal mitral and aortic valves. Two dimensional echocardiography provides excellent morphological information about the relationship between the accessory mitral valve tissue and the mitral and aortic valves, respectively. Accurate preoperative evaluation by two-dimensional echocardiography facilitates the successful surgical management of this rare condition.

Dissection of the descending aorta after balloon angioplasty of native coarctation.

A 12-year-old girl underwent successful balloon angioplasty for a waist-like native coarctation. The balloon size/coarctation diameter ratio was 3.3. Postdilatation angiography showed a small aneurysm at the coarctation site. On frequent review the patient remained symptom-free and normotensive. Recatheterization was performed 14 months after balloon aortoplasty, when angiography revealed a massive aortic dissection extending from the origin of the left subclavian artery to both iliac arteries. She underwent partial replacement of the thoracic aorta. Balloon angioplasty of a narrow waist-like native coarctation may lead to extensive wall dissection and should be considered critically.

Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis.

OBJECTIVE: The aim of this study was to evaluate the effects of inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. METHODS: Inhaled nitric oxide (mean 4.1 +/- 0.7 ppm, 1.5 to 10 ppm) was administered in 13 patients (mean age 5.6 +/- 1.6 years, 1.5 to 17 years) with critical pulmonary perfusion (central venous pressure > 20 mm Hg or transpulmonary pressure gradient > 10 mm Hg) in the early postoperative period after total cavopulmonary connection (n = 9) or after bidirectional Glenn anastomosis (n = 4). RESULTS: In patients after total cavopulmonary connection inhaled nitric oxide therapy decreased central venous pressure by 15.3% +/- 1.4% (p = 0.0001) and transpulmonary pressure gradient by 42% +/- 8% (p = 0.0008) and increased mean systemic arterial and left atrial pressures by 12% +/- 3.6% (p = 0.011) and 28% +/- 8% (p = 0.007), respectively. Arterial and venous oxygen saturations improved by 8.2% +/- 1% (p = 0.005) and 14% +/- 4.3% (p = 0.03), respectively. In patients after bidirectional Glenn anastomosis inhaled nitric oxide therapy resulted in a decrease of central venous pressure by 22% +/- 1% and of the transpulmonary pressure gradient by 55% +/- 6% and improved arterial and venous oxygen saturations by 37% +/- 29% and 11% +/- 3%, respectively. Mean systemic arterial and left atrial pressures remained nearly unchanged. No toxic side effect was observed in any patient. CONCLUSION: Inhaled nitric oxide may play an important role in the management of transient critical pulmonary perfusion caused by reactive elevated pulmonary vascular resistance in the early postoperative period after Fontan-type operations and bidirectional Glenn anastomosis.

Catheter-directed thrombolysis with recombinant tissue plasminogen activator for acute pulmonary embolism after fontan operation.

We report the case of a 3-year-old girl who presented with near-lethal pulmonary thrombembolism 3 weeks after an uneventful Fontan operation. Complete occlusion of the left lower lobe pulmonary artery had occurred together with a cerebral infarction. Recombinant tissue plasminogen activator (rt-Pa) was used for thrombolysis because of its short half-life and its clot-selective properties. To further minimize the systemic effects of rt-PA, local catheter-directed lysis was performed. A prolonged course of low-dose rt-PA therapy achieved complete lysis without side effects.

Perinatal manifestations of idiopathic long QT syndrome.

A neonate who had presented with sustained irregular heart rate during labor was found to have QT prolongation and repetitive polymorphic ventricular tachycardia (torsades de pointes) postnatally. Propranolol and propafenone successfully controlled the ventricular arrhythmias. Follow-up electrocardiograms and Holter records show persistent QT prolongation, bizarre T waves, and intermittent episodes of T wave alternans. On propranolol monotherapy the boy is thriving and completely free of ventricular arrhythmias. In the rare case of long QT syndrome in the neonate, early detection and therapy are mandatory to prevent ventricular arrhythmias and sudden death.

Extralobar sequestration with unusual arterial supply and venous drainage.

We present a case of extralobar pulmonary sequestration between the left lower lobe and diaphragm with an unusual arterial blood supply and venous drainage. Angiography revealed a large systemic artery arising from the left subclavian artery. The venous return paralleled this anomalous artery and drained into the left subclavian vein. This case illustrates the wide anatomic variability of such complex bronchovascular anomalies. Careful preoperative evaluation of both the arterial supply and venous drainage is important to avoid intraoperative complications. Angiography provides clear definition of these abnormal vascular structures, which is essential for appropriate therapeutic management.

TMJ arthrocentesis. A conservative surgical alternative.

TMJ arthrocentesis entails placing two needles into the joint space for purposes of lysis and lavage via hydraulic distension. For arthropathy patients unresponsive to nonsurgical care, arthrocentesis is a simplified alternative to the most common arthroscopic surgical procedure. It is minimally invasive and may be performed in the office with comparable success and diminished morbidity.

[Balloon dilatation of postoperative and congenital aortic isthmus stenoses]. / Ballondilatation postoperativer und nativer Aortenisthmusstenosen.

From July 1987 to December 1992, 52 patients underwent balloon-angioplasty of aortic coarctation at three units of pediatric cardiology in Austria (Graz = 35 patients, Innsbruck = 15 patients, and Vienna = 2 patients). 35 patients had postoperative and 17 had native coarctation. Mean age at intervention was 7 10/12 years with 2 patients under 1 year and 5 patients over 18 years old. The mean relation balloon diameter-coarctation diameter was 2.6 +/- 0.9. The blood pressure gradient between upper and lower extremities decreased from a mean of 44 +/- 16 mm Hg to 15 +/- 13 mm Hg (p < 0.0001). The diameter of the stenosed segment was increased from 5 +/- 3 mm to 8.5 +/- 3.5 mm (p < 0.0001). Native coarctation showed a significantly better result in respect to decrease of the gradient (36 +/- 12 mm Hg) than did postoperative coarctations (25 +/- 19 mm Hg) (p < 0.03). 13 patients did not respond adequately to angioplasty. 10 patients out of this group had tubular narrowings and belonged to the group of postoperative coarctations, whereas localized stenoses in native coarctations gave the best results. Localized wall irregularities were found in 4 patients with native coarctation. Balloon angioplasty of postoperative and native coarctations in childhood and adolescence is a secure and effective means of treatment and should be considered as therapy of first choice. The best results can be found in the group of the circumscript type of stenoses in native coarctation, whereas long and tubular stenoses in the group of postoperative coarctations give less satisfying results.

[Total cavopulmonary connection in complex heart defects with a single functional ventricle]. / Totale kavopulmonale Konnektion bei komplexen Herzfehlern mit funktionell singulärem Ventrikel.

Nineteen children aged between 1.5 and 9.8 years (10 patients younger than 4 years) underwent total cavopulmonary connection (TCPC) for complex congenital heart defects other than tricuspid atresia. Diagnoses included double inlet left ventricle (n = 11), transposition of the great arteries with a large ventricular septal defect (n = 4), double outlet right ventricle (n = 2), congenitally corrected transposition of the great arteries with a large ventricular septal defect (n = 1) and criss-cross heart (n = 1). Two patients had situs ambiguous with polysplenia syndrome and four patients had anomalous systemic venous return. Thirty-six palliative procedures were performed in 15 patients before TCPC. The patients were selected according to the selection criteria of Choussat and Fontan. Four patients (21%) died between the 1st and 12th postoperative day because of low cardiac output syndrome (n = 2), supraventricular tachycardia unresponsive to treatment (n = 1), and cerebral edema (n = 1). In one patient take down of TCPC was performed for persistent low cardiac output syndrome. Four of these five patients were younger than 4 years old and had one or more additional risk factors. Postoperative complications were persistent pleural or pericardial effusions (n = 4), transient supraventricular tachycardia (n = 2), and low cardiac output syndrome (n = 1). There was no late death. Follow-up was available in 12 patients. At a mean follow-up of 15 +/- 9 months, nine patients are in NYHA-functional class I, two patients in class II, and one patient in class III. Postoperative cardiac catheterization revealed satisfactory results in seven of eight patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Cloning of Genes Involved in the Synthesis of Pyrrolnitrin from Pseudomonas fluorescens and Role of Pyrrolnitrin Synthesis in Biological Control of Plant Disease.

A soil isolate of Pseudomonas fluorescens (BL915) was shown to be an effective antagonist of Rhizoctonia solani-induced damping-off of cotton. Investigation of the biological basis of this antagonism revealed that the strain produces pyrrolnitrin, a secondary metabolite known to inhibit R. solani and other fungi. Mutants of strain BL915 that did not produce pyrrolnitrin and did not suppress damping-off of cotton by R. solani were generated by exposure to N-methyl-N' -nitro-N-nitrosoguanidine. A gene region that was capable of restoring pyrrolnitrin production to the non-pyrrolnitrin-producing mutants and of conferring this ability upon two other P. fluorescens strains not otherwise known to produce this compound or to be capable of suppressing damping-off caused by R. solani was isolated from strain BL915. The non-pyrrolnitrin-producing strains (mutants of BL915 and the other two P. fluorescens strains) which synthesized pyrrolnitrin after the introduction of the gene region from strain BL915 were also shown to be equal to strain BL915 in their ability to suppress R. solani-induced damping-off of cotton. These results indicate that we have isolated from P. fluorescens BL915 a gene(s) that has a role in the synthesis of pyrrolnitrin and that the production of this compound has a role in the ability of this strain to control damping-off of cotton by R. solani.

Atypical presentation of Kawasaki disease in an infant.

A 7-month-old male infant with clinical symptoms of severe toxic shock syndrome died on day 9 of illness. At autopsy, demonstration of coronary vasculitis together with thrombosis of the left coronary artery revealed the true diagnosis of atypical Kawasaki disease. The marked similarity in many clinical features makes the distinction between these two diseases difficult when atypical clinical presentation of Kawasaki disease is present.

Augmenting the maxillary sinus for implants: report of 27 patients.

A method for restoring the posterior portion of the maxilla with a simultaneous composite alloplastic sinus lift graft and placement of press-fit implants is presented. Sinus grafting is needed because of minimal remaining alveolar bone. The composite graft material uses a combination of demineralized freeze-dried cortical bone and Interpore 200 hydroxyapatite. Since 1987, 45 sinus grafts with simultaneous placement of 111 root-form implants have been performed for 27 patients. None of the 76 restored implants have been lost. Complications were encountered in 2 patients (4 sinuses) who were heavy smokers. Antibiotics were administered, symptoms subsided, and the implants integrated.