Prenatal echocardiographic assessment of right aortic arch.

OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern in relation to the trachea, and to establish whether the echocardiographic 'V-shaped' or 'U-shaped' appearance of the junction between the DA and aortic arch (AA) in the fetal upper mediastinal view is sufficiently accurate for assessment of fetal AA anatomy. METHODS: This was a retrospective study of pregnancies with a prenatal diagnosis of fetal RAA that had postnatal confirmation of AA anatomy, referred to our tertiary center during 2011-2017. Prenatal and postnatal medical records, including echocardiographic and computed tomography (CT)/magnetic resonance imaging (MRI) scan reports, were reviewed, and cardiac and extracardiac abnormalities and the results of genetic testing were recorded. RESULTS: Of 55 consecutive pregnancies with a prenatal diagnosis of fetal RAA, six were lost to follow-up, one was terminated and three were excluded due to lack of postnatal confirmation of AA anatomy. Of the remaining 45 pregnancies, AA anatomy was assessed postnatally by CT in 39, by MRI in one and by direct examination at cardiac surgery in five. A U-shaped appearance was found in 37/45 (82.2%) patients, all of which had a complete vascular ring (CVR). Of these 37 patients, on postnatal confirmation, 21 (56.8%) had RAA with Kommerell's diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11 (29.7%) had a double AA (DAA), four (10.8%) had RAA with Kommerell's diverticulum, LPDA and mirror-image (MI) branching (RAA/LPDA/MI), and one (2.7%) had RAA with Kommerell's diverticulum, LPDA and aberrant left innominate artery (ALIA) (RAA/LPDA/ALIA). A V-shaped appearance was found in 3/45 (6.7%) patients, all of which had RAA with right DA not forming a CVR and MI branching. In the 5/45 (11.1%) fetuses with neither U- nor V-shaped appearance, RAA with left anterior DA arising from the left innominate artery and MI branching, not forming a CVR, was found. Twelve (26.7%) fetuses had a congenital heart defect (CHD). RAA forming a CVR (U-shaped appearance) was associated with a septal defect in 6/37 (16.2%) fetuses, while RAA not forming a CVR (V-shaped appearance or no U- or V-shaped appearance) was associated with major CHD in 6/8 (75.0%) fetuses. CONCLUSIONS: In fetuses with RAA, V-shaped appearance of the junction between the DA and AA indicates only that the transverse AA and DA run together on the same side of the thorax (trachea) while a U-shaped appearance is always a sign of a CVR. Among fetuses with a CVR, RAA/LPDA/MI is more frequent than described previously. Finally, RAA forming a CVR is not usually associated with complex CHD, as opposed to RAA not forming a CVR. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Shelhigh No-React porcine pulmonic valve conduit: a new alternative to the homograft.

BACKGROUND: The Shelhigh No-React pulmonic valve conduit is a new porcine conduit that is glutaraldehyde-treated and detoxified using a proprietary heparin process. In our institution it has been implanted in 25 patients. The aim of this present contribution is to evaluate the short-term follow-up after its implantation. METHODS: From November 1997 to August 1999, 25 patients (mean age, 20.2 years; range, 0.6 to 28.3 years) were operated on using this conduit. Seventeen patients underwent a Ross procedure for aortic valve disease, with the conduits implanted in anatomic position; 6 patients underwent right ventricular outflow tract reconstruction; 2 patients underwent the Rastelli operation. The follow-up was complete. Preoperative and postoperative two-dimensional echocardiography data were collected. RESULTS: There were two non-conduit-related deaths. Two conduits needed to be exchanged because of an increase in the gradient. Overall, all patients were improved in terms of New York Heart Association class. Comparison of preoperative and postoperative two-dimensional echocardiography gradient showed significant improvement. At the 30-month follow-up, no calcification was seen on the explanted conduits or on the two-dimensional echocardiography, although many of the patients are children. CONCLUSIONS: The Shelhigh conduits seem to be an alternative to homograft especially in infants. These experiences are preliminary, and longer follow-up is required.

Fast-track congenital heart operations: a less invasive technique and early extubation.

BACKGROUND: Many novel techniques have been described for "minimally invasive" congenital cardiac operations to achieve an improved cosmetic result. There is little information on incorporation of such techniques into fast-track congenital heart operations. METHODS: We have developed an approach to fast-track congenital heart operations, which includes a cosmetic approach for repair of congenital heart defects without sacrificing adequate exposure or requiring specialized equipment, along with a simple approach to intraoperative anesthetic management that allows extubation in the operating room. The heart is exposed through a short midline skin incision and a full median sternotomy. The conventional technique of cannulation is performed. Between October 1997 and January 1999, 88 patients were operated on with this method. Cardiac anomalies included simple and complex ostium secundum atrial septal defect, sinus venous atrial septal defect, partial atrioventricular septal defect, simple and complex ventricular septal defect, and bicuspid aortic valve stenosis. RESULTS: There were no operative or late deaths. The majority of patients were extubated in the operating room or within 2 hours of operation. No patient underwent reoperation and the mean length of hospital stay was 3.9 days. Sternal instability or wound infection were not observed. CONCLUSIONS: We believe that our approach to fast-track congenital heart operation is safe and effective. The surgical technique provides good exposure and has excellent cosmetic results. Moreover, it is easy to learn and, if necessary, the surgeon can quickly gain direct access to the heart. The anesthetic management facilitates early tracheal extubation and a shorter duration of postoperative stay.

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt.

A 21-year-old white woman, born with a univentricular heart, had undergone staged procedures before Fontan correction. She then began to develop edema, protein-losing enteropathy, and ascites refractory to diuretic therapy. Cardiac angiography showed a patent right Blalock-Taussig shunt, with turbulent cavopulmonary circulation. After undergoing an unsuccessful attempt at coil embolization she then underwent shunt ligation, with resolution of symptoms and normalization of protein levels. This report draws attention to the importance of cavopulmonary laminar flow to prevent the development of protein-losing enteropathy.

Intrapericardial teratoma in a newborn: a case report.

Intrapericardial teratomas are unusual tumors that often cause respiratory distress and might be lethal in the newborn. The purpose of this article is to present the clinical and pathological findings in a 12-day-old male successfully treated for a big intrapericardial teratoma. Given that the diameter of this tumor is generally related to the age at the time of diagnosis, the rarity of our case is the presence of a huge intrapericardial teratoma in a newborn. The surgical resection was lifesaving.

[The conversion of a modified Fontan procedure to a total extracardiac cavo-pulmonary conduit. The Medico-Surgical Cardiology Group]. / Conversione della procedura di Fontan modificata a connessione cavopolmonare totale extracardiaca. Raggrupamento di Cardiologia Medico Chirurgica.

BACKGROUND: After a modified Fontan procedure with atriopulmonary or atrioventricular conduit, some patients present stress intolerance, supraventricular arrhythmia, recurrent pleuropericardial or ascitic effusions, and protein-losing enteropathy, all of which are signs that the previous procedure has failed. The aim of this study was to evaluate the midterm outcome after surgical therapy for this condition. MATERIAL AND METHODS: Between August 1994 and December 1997, nine patients (6 males and 3 females), age 10 to 39 (mean 21.5) years, underwent conversion of previous modified Fontan procedure to total extracardiac cavo-pulmonary connection. Time from the previous procedure was 6 to 18 years (mean 10). Diagnosis was tricuspid atresia with pulmonary stenosis (n = 2), double-inlet left ventricle and concordant ventriculoarterial connection (n = 3), double-inlet left ventricle and discordant ventriculoarterial connection (n = 3), Holmes heart (n = 1). Nine patients presented decreased stress tolerance, seven had arrhythmia, five had pleuropericardial effusions and two had protein-losing enteropathy. In all but one patient, right atrial pressure was higher than 15 mmHg, while in six patients the cardiac index was less than 2 l/min/m2. A polytetrafluoroethylene non-valved conduit was interposed between the inferior vena cava and the right pulmonary artery for conversion in all patients. A bidirectional cavo-pulmonary anastomosis (modified Glenn) was associated in all patients. Evaluation was done by NYHA Class and by an arbitrary score assigned to patients based on 7 parameters. RESULTS: There was no perioperative mortality. All patients were clinically improved at a mean follow-up of 24 months (range: 3 to 46). No patient had effusions, and the arrhythmias disappeared in 4 patients and were controlled by medical therapy in one. The two patients with protein-losing enteropathy improved markedly within 30 days and the score dropped below 10 points. CONCLUSIONS: The conversion of the modified Fontan procedure to total extracardiac cavo-pulmonary connection improves clinical condition by decreasing the right atrium-pulmonary gradient and right atrial preload, and by providing a laminar cavo-pulmonary flow without any need for intracardiac anastomoses. This procedure should be undertaken early in this subset of patients, before ventricular failure ensues.

[The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies]. / Ruolo dell'anastomosi cavo-polmonare bidirezionale nella correzione e palliazione delle cardiopatie congenite complesse.

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.

Aortic pulmonary autograft implant: medium-term follow-up with a note on a new right ventricular pulmonary artery conduit.

BACKGROUND: The Ross operation has been applied to various aortic valve pathologies, particularly when somatic growth is an issue. However, associated cardiac disease and technical problems may limit its use with regard to associated procedures and issues of right ventricular outflow reconstruction. MATERIALS AND METHODS: From December 1992 to March 1998, 24 patients underwent aortic pulmonary autograft implantation. There were 14 males and 10 females, 15+/-10 years of age (mean +/- SD) (range 1 to 50 years), weighing 42.8+/-20 kg (mean +/- SD) (range 8 to 78 kg). Aortic insufficiency was present in 15 (62.5%) patients, stenosis in 8 (33.3%) patients, and valvar stenosis associated with left ventricular outflow tract obstruction in 1 (4.1%) patient. Etiology was rheumatic in 17 patients and congenital in 7. The Ross procedure was accompanied by a partial-Konno left ventricular outflow enlargement in one patient, and mitral valve annuloplasty, mitral commissurotomy, and tricuspid valve replacement in three other patients, respectively. The right ventricular outflow was reconstructed with a valved pulmonary homograft in 14 patients and with a Shelhigh No-React porcine pulmonary conduit in 10 patients. Evaluation was done by New York Heart Association (NYHA) Class and by echocardiography at a follow-up of 22.8+/-24 months (mean +/- SD) (range 3 to 63 months). RESULTS: There were no operative mortalities and no postoperative arrhythmias. One (4.1%) patient required intra-aortic balloon pump (IABP) support for 3 days, one (4.1%) patient died 2 years later of probable arrhythmia, and one (4.1%) patient required mechanical aortic valve replacement 2 years later for severe autograft insufficiency. Left ventricular ejection fraction was unchanged (preoperative 62.4%+/-30%, postoperative 64.2%+/-30% [mean +/- SD], [p = NS]) and no significant gradient was documented by echocardiographic Doppler in the right and left ventricular outflow tracts. The aortic insufficiency scale decreased from a mean of 3.9+/-0.2 to a mean of 1+/-0 (p < 0.01). NYHA Class decreased to I in all patients, from III (10) and II (14). CONCLUSIONS: The pulmonary autograft in the aortic position is suitable for aortic valve replacement in pediatric and adult patients with good medium-term results and in patients with rheumatic etiology, and it provides a desirable solution in the presence of associated pathologies, such as left ventricular tract obstruction or associated multivalvular disease. The development of new means of right ventricular outflow reconstruction must parallel the progress achieved for the left side.

Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options.

BACKGROUND: An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure. METHODS: From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter. RESULTS: There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy. CONCLUSIONS: Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve.

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Combined mustard and Rastelli operations. An alternative approach for repair of associated anomalies in congenitally corrected transposition in situs inversus [I,D,D].

We report on two patients with congenitally corrected transposition of the great arteries in situs inversus who underwent successful anatomic repair of associated cardiac anomalies--ventricular septal defect and pulmonary outflow tract obstruction. Surgical intervention was influenced by the recently introduced technique of Ilbawi and colleagues in cases of congenitally corrected transposition in situs solitus. Principles of the correction are as follows: (1) patch redirection of venous flows at the atrial level through an incision in the left-sided right atrium; (2) patch closure of the ventricular septal defect through a right ventriculotomy, baffling the left ventricle to the aorta; and (3) valved conduit interposition between the right ventricle and the pulmonary artery. If the ventricular septal defect is restrictive, it can be safely enlarged by extensive resection of the anterosuperior border, because of the posteroinferior location of the bundle of His in this anomaly. With this technique, the left ventricle and the native mitral and aortic valves are recruited for systemic work, with intrinsic long-term advantages. It is, however, a complex procedure involving substantial use of prosthetic material. More experience is necessary to establish whether this approach is superior to currently available physiologic repairs.

"Acquired" subvalvular aortic stenosis after repair of a ventricular septal defect.

Of 353 children who underwent surgical repair of a congenital heart defect, including closure of a ventricular septal defect (VSD), 12 patients (four with tetralogy of Fallot, five with a VSD, and three with a double-outlet right ventricle) developed subaortic stenosis, which was diagnosed one to six years after the surgical procedure. Five patients required surgical treatment of the subaortic stenosis, and one required percutaneous balloon angioplasty. Postsurgical subaortic stenosis appears to be an uncommon progressive acquired disease.

Surgical treatment of anomalous origin of the left coronary artery in infancy.

Two cases of anomalous origin of the left coronary artery (LCA) from the pulmonary artery (PA) are reported in which early surgical correction was achieved by an end-to-end anastomosis of the left subclavian artery (LSA) to the LCA, with cardiopulmonary bypass and deep hypothermia. The clinical improvement was rapid and the left ventricular function recovered almost completely. We recommend this type of surgical correction, early in life, for infants in severe cardiac failure and anomalous origin of the LCA.