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Graphical abstract, Highlights • PVL after TMVR with a Tendyne device can result in severe intravascular hemolysis. • SAM of the mitral valve can cause anterior PVL following Tendyne TMVR. • TAVR can relieve LVOT obstruction caused by SAM after Tendyne TMVR. • Pre-, peri-, and postprocedural imaging is key in decision making.

Summary Background Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. Aims To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. Methods Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. Results The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r = 0.98 for both) and a low mean bias (0.71 mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between −0.9 and 2.3 mm and between −0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P = 0.13). All computed tomography scans were performed without sedation. Conclusions Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

This Viewpoint presents and discusses the development of the first core principles and standards for effective, personalised care of children living with complex care needs in Europe. These principles and standards emerged from an analysis of data gathered on several areas, including the integration of care for the child at the acute-community interface, the referral-discharge interface, the social care interface, nursing preparedness for practice, and experiences of the child and family. The three main principles, underpinned by a child-centric approach, are access to care, co-creation of care, and effective integrated governance. Collectively, the principles and standards offer a means to benchmark existing services for children living with complex care needs, to influence policy in relation to service delivery for these children, and to provide a suite of indicators with which to assess future service developments in this area.

Major congenital or acquired heart disease in neonates presents with cyanosis, hypoxia, acute circulatory failure or cardiogenic shock. Antenatal diagnosis is made in up to 50% but heart disease is unanticipated in the remainder. The presence of significant heart disease in premature infants is also frequently not suspected at first; in general, whatever the underling cardiac anomaly, the clinical condition is worse, deteriorates more quickly and carries a poorer prognosis in premature and low birth weight infants. Although congenital cardiac malformations are the most likely, other important cardiac disorders are encountered. In general initial treatment options, often without a precise diagnosis, include diuretics, prostin, catecholamines, phosphodiesterase inhibitors, ventilation and occasionally ECMO but the key to successful treatment remains the correct diagnosis. Many conditions will only show significant improvement with treatment by the interventional cardiologist or cardiac surgeon.

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

To assess right ventricular function in adults and children with Ebstein's anomaly.Prospective study.Tertiary referral centre.Fifteen patients (8 adults and 7 children) with Ebstein's anomaly and 14 healthy controls.Transthoracic echocardiography was performed in all. Right ventricular function was assessed from long axis M-mode recordings of the right atrio-ventricular free wall. Total systolic excursion as well as peak shortening and lenghtening rates of the right ventricle were measured.Children and adult patients with Ebstein's anomaly differed in terms of age at diagnosis, the adult group having been diagnosed later 19.8+/-15.8 vs. 5.9+/-6.2 years, P0.05. Measures of right ventricular long axis function in children with Ebstein's anomaly showed a significantly reduced systolic excursion 1.4+/-0.4 vs. 2.4+/-0.53 cm, P0.05 and peak lenghtening rate; early 8.04+/-4.3 vs. 11.8+/-2.4 cm/s and late 6.14+/-3.6 vs. 10.6+/-4.3 cm/s, P=0.05 compared to controls. In contrast, measurements of right ventricular long axis function in adults with Ebstein's anomaly showed no significant difference when compared to the control group. Transtricuspid Doppler flow velocities were not different between patient's groups and corresponding controls.The right ventricle assessed by this simple, non-invasive technique reveals a significantly reduced systolic and diastolic function in children with Ebstein's malformation compared to controls but a 'normal' right ventricular function comparable to controls in adult patients. Significant right ventricular dysfunction in children with Ebstein's anomaly could account for their earlier presentation. Long term follow up of the right ventricular abnormalities is needed in such children to discover more about the natural history of the disease.

We report the case of a 12-year-old boy with a hypoplastic retroesophageal circumflex right-sided cervical aortic arch and coarctation. After the incidental finding of a heart murmur when the boy was 9 years old, cardiac magnetic resonance showed a right-sided cervical aortic arch, hypoplastic transverse arch, and separate origin of the left common carotid, right common carotid, right vertebral, and right subclavian arteries. The left subclavian artery arose from the proximal descending aorta next to the coarctation. An extra-anatomical ascending to descending aorta tube graft was inserted through a right lateral thoracotomy with good results.

A 66-year-old man was scheduled for percutaneous balloon valvuloplasty of pulmonary valvular stenosis under general anesthesia in the cardiac catheterization laboratory. The patient had a significant history of cardiorespiratory disease. In 1944, his family physician detected a heart murmur, and in 1955, the patient was referred to a cardiologist and told he had “a narrowed valve.” The patient was then lost to follow-up, and his old hospital records were lost. The patient claimed to have been asymptomatic until he sustained an inferior myocardial infarction in 1999 and was referred to this hospital for further investigations. On admission, the patient admitted to smoking 15 cigarettes per day until his myocardial infarction, and his exercise tolerance was reduced to less than 500 meters during the last few years. His medications were aspirin and atenolol. On examination, the patient was noted to be cyanotic, had clubbing of the fingers, had a raised jugular venous pressure, and had bilateral basal crepitations on chest auscultation. The patient had a soft ejection systolic murmur with a normal second heart sound, and there was no right ventricular heave. Further investigations revealed polycythemia (hemoglobin, 19 g/dL; hematocrit, 57.3%), mild renal impairment (serum creatinine, 143 mmol/ L), and arterial blood gases and lung function testing as shown in Tables 1 and 2. A chest radiograph was normal, and a 12-lead electrocardiogram showed T-wave inversion in leads I through III and no evidence of right ventricular hypertrophy. At cardiac catheterization, the patient was found to have moderate left ventricular dysfunction, with a mild stenosis of the left anterior descending coronary artery and complete occlusion of the dominant right coronary artery. There was a severe pulmonary valve stenosis with a peak-to-peak systolic gradient of 90 mmHg (Fig 1). A small atrioseptal defect or patent foramen ovale was noted and confirmed with transesophageal echocardiography. Transesophageal echocardiography did not show any evidence of right or left ventricular dysfunction, and left ventricular ejection fraction was calculated to be 60%. Oxygen saturations and pressure measurements are shown in Tables 3 and 4. It was decided that the pulmonary stenosis should be treated by percutaneous balloon valvuloplasty. Standard anesthetic monitoring was applied, including a 5‐lead electrocardiogram and direct arterial blood pressure measurement via a 20G radial artery catheter inserted under local anesthesia. Pulse oximetry in air showed a saturation (SpO2) of 92%. After preoxygenation (SpO2 increased to 100%), anesthetic induction consisted of fentanyl, 100 g; etomidate, 16 mg; and vecuronium, 8 mg. After endotracheal intubation, the lungs were ventilated with oxygen, air, and isoflurane, 0.8% to 1.4%, for maintenance of anesthesia. The right internal jugular vein was cannulated with 2 long 14G catheters for monitoring of central venous pressure and easy administration of drugs. During the procedure, 1000 mL of lactated Ringer’s solution was infused. Anesthesia was uneventful, and the angiographic findings compared well with the previous measurements. Balloon pulmonary valvotomy using a 25 mm 40 mm balloon resulted in the peak-to-peak gradient falling to 12 mmHg (Figs 2 and 3, Table 5). The patient’s other cardiovascular parameters were unchanged, and PaO2 improved immediately to 9.65 kPa. The procedure time was 15 minutes. The small atrial septal defect was of little or no significance, and no further procedures were carried out. Neuromuscular blockade was reversed with glycopyrrolate, 500 g, and neostigmine, 2.5 mg, and the patient’s lungs were ventilated with oxygen until adequate spontaneous ventilation was restored and the patient’s trachea could be extubated. Total anesthetic time was 45 minutes. The patient was transferred to the postanesthesia care unit to await transfer to the ward. Within 5 minutes of admission to the postanesthesia care unit, the patient developed a tachycardia (120 beats/min), his respiratory rate increased to 30 breaths/min, and SpO2 decreased to 85%, despite supplemental oxygen by facemask.

Survival before definitive operations was studied in 191 infants with double-inlet ventricle presenting before 1 year of age (1973 to 1988, median follow-up 8.5 years). The morphologic spectrum was broad, with a great prevalence of associated lesions. The actuarial survival rate before definitive repair was 57% at 1 year, 43% at 5 years, and 42% at 10 years, worse than prior reports because of the younger age at entry into our series. Analysis of univariate risk factors established that right atrial isomerism (18% of the group, relative risk 2.9), common atrioventricular orifice (42%, 2.0), pulmonary atresia (20%, 3.4), obstruction of the systemic outflow tract (18%, 2.5), and extracardiac anomalous pulmonary venous connection (13%, 3.1) were strongly associated with poorer survival. Pulmonary stenosis (40%, 0.35), balanced pulmonary blood flow (9%, 0.40), and presentation at an older age (33%, 0.42 to 0.18) were beneficial (p

Thirteen patients have undergone surgical treatment because of subaortic obstruction in hearts with a univentricular atrioventricular connection. Nine patients underwent surgical enlargement of the ventricular septal defect and four patients had construction of an aortopulmonary anastomosis and closure of the pulmonary trunk (the Damus-Kaye-Stansel procedure). Two patients undergoing enlargement of the septal defect and two having the Damus-Kaye-Stansel procedure also had a modified Fontan procedure. One patient had complete atrioventricular dissociation after direct enlargement of the ventricular septal defect, which necessitated insertion of an epicardial pacemaker. One patient died within 30 days of the operation after enlargement of the defect and two patients after the Damus-Kaye-Stansel procedure. There was one late death, occurring in a patient who underwent enlargement of the ventricular septal defect Ten patients have subsequently undergone conventional cardiac catheterization and angiography or transcutaneous Doppler flow studies to assess the relief of the subaortic obstruction. The result has been satisfactory in all. Because of this experience, we now recommend direct surgical enlargement of the restrictive ventricualr septal defect for direct relief of subaortic stenosis occurring with a univentricular atrioventricular connection to a dominant left ventricle, inasmuch as it appears to be hemodynamically effective with a low operative mortality and morbidity The Damus-Kaye-Stansel procedure can also have a role in relieving subaortic stenosis when the atria are connected to a dominant right ventricle.

Twenty asymptomatic school children who had undergone Mustard's operation for simple complete transposition (concordant atrioventricular and discordant ventriculo-arterial connexions) were catheterised electively 6–13 years later. The studies were carried out under general anaesthesia in air and in 100% O 2 . Oxygen consumption was measured and end-tidal gases were monitored using respiratory mass spectrometry. There was significant left ventricular outflow tract obstruction in 2 patients. Cardiac output in air was normal in 15 and decreased in 5 patients. The pulmonary vascular resistance was normal in 18 of 19 cases, but grossly elevated in one patient. Baffle dysfunction was present in 11 patients: 10 with important gradients between the venous pathways and the systemic venous atrium, and 5 with a leak identified either by a left-to-right shunt or by the course of the catheter. Balloon dilatation was attempted in the inferior caval venous channel in 6 and in the superior caval venous channel in 2. Mean gradient before the dilatation fell after the procedure. No pulmonary venous obstruction was identified. Even in this group of children selected as asymptomatic, approximately half had a detectable haemodynamic abnormality.

We analyzed, using a sequential segmental approach, 32 cases of double-inlet ventricle to assess the feasibility of surgical "correction" by either ventricular septation or a modified Fontan procedure. Twenty-two hearts had two atrioventricular valves, connected to a left ventricle in 19, a right ventricle in two, and a solitary indeterminate ventricle in one. Septation was possible in only 13. In contrast, the Fontan procedure seemed feasible in 20. The remaining 10 specimens had double inlet via a common valve to the left ventricle in two, the right ventricle in six, and an indeterminate ventricle in two. Seven of these had right atrial isomerism. Ventricular septation was not considered a possibility in these hearts. The Fontan procedure combined with atrial septation was a possibility in seven cases. From the morphologic stance, although the modified Fontan procedure seemed suitable in most cases, a significant number of hearts with two atrioventricular valves were suitable for ventricular septation.

Eighty-three patients underwent surgical correction of total anomalous pulmonary venous connection (TAPVC) between 1973 and 1986. There were 46 boys and 37 girls. Median age at operation was 60 days (1 to 240) and median weight 3.9 kg (1 to 22). The anatomic types encountered included infracardiac connection (16 patients), supracardiac connection (32) and pulmonary venous drainage connected directly to the coronary sinus (27). Mixed anomalous drainage or pulmonary venous return connected directly to the right atrium occurred in 8 patients. Diagnosis was established by cardiac catheterization and angiography (56 patients), clinical examination (3) and cross-sectional echocardiography alone in 24 of the last consecutive 28 patients. Pulmonary hypertension was present in 26 (55%) of those who underwent cardiac catheterization. The median pulmonary vascular resistance was 4.2 units/m 2 (body surface area) for all the patients, whereas in those with infracardiac pulmonary venous connection the median value was 10 units/ m 2 . The median interval between admission and operation was 72 hours. Surgical correction was performed using profound hypothermia and circulatory arrest in 68; for the remainder, conventional cardiopulmonary bypass with profound to moderate hypothermia was used. Ten patients developed 1 or more pulmonary hypertensive crises during the early postoperative period. These were diagnosed in 8 by direct pulmonary artery pressure measurement and in 2 by clinical examination. Late reoperation was necessary in 6 patients (10%). Analyses of risk factors for 30-day survival for all patients showed that the risk of early death was associated with the type of anomaly (infradiaphragmatic), occurrence of pulmonary hypertensive crises, year of the operation, sex of the patient and presence of preoperative congestive heart failure. The risk of early death was decreased when the method of diagnosis was cross-sectional echocardiography alone. The 30-day hospital mortality from 1975 to the present was 8.5%. Six patients (10%) died later than expected. Actuarial survival of patients with infradiaphragmatic drainage was 60% at 72 months and the remainder had an 88% survival at 80 months. The whole group had an actuarial survival of 75% at 84 months.

In the accompanying paper [l], Wenink and Otter&amp from the University of Leiden continue their fascinating researches into the developmental origins of hearts with no direct communication between one atrial chamber and the ventricular mass. Conventionally termed “atrioventricular valve atresia”, these hearts have been a continuing source of controversy and contention amongst morphologists. One argument, for the most part non-productive and fomented in no small way by ourselves, has been whether such hearts can be considered “univentricular” [2,3]. Another argument, where we have expressed opinions at variance with our Leiden colleagues, devolves on the appropriateness of the terms “tricuspid” and “mitral” atresia for these various lesions. The needed resolution of the first of these problems was provided when we showed [4,5] that valve “atresia” could be produced either by an imperforate valve membrane or absence of one atrioventricular connexion (Fig. 1) and that the feature that linked the majority of these hearts to those with double inlet ventricle was the univentricular nature of their atrioventricular connexion rather than their ventricular mass (Fig. 2). Such considerations show that, taken together, there is considerable morphological heterogeneity to be found in hearts which the clinician would diagnose as having “ tricuspid atresia”. Almost all clinicians would likely diagnose a patient as having “ tricuspid atresia” when the systemic venous return is unable directly to reach the ventricular mass. Instead, the systemic venous blood must cross the atrial septum from the morphologically right atrium and mingle with the pulmonary venous return in the morpho

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.