Long-term follow-up and sex differences in adults operated for tetralogy of Fallot.

OBJECTIVE: Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. METHODS: We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. RESULTS: We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). CONCLUSIONS: Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment.

Is experienced pregnancy in women with repaired tetralogy of Fallot related to diffuse myocardial fibrosis?

OBJECTIVES: To assess the impact of pregnancy on cardiac function and fibrosis by cardiovascular magnetic resonance (CMR) in patients with repaired Tetralogy of Fallot (rToF). BACKGROUND: CMR T1 mapping can assess diffuse myocardial fibrosis which is associated to adverse clinical outcomes. Right ventricular (RV) accelerated remodeling is reported in rToF women with experienced pregnancy. METHODS: We included rToF women from the national registry of congenital heart disease to perform CMR, assessing functional data, T1 mapping/ extracellular volume fraction (ECV). The results including clinical data were compared between women with experienced pregnancy vs non-experienced pregnancy and healthy individuals. RESULTS: Fifty rToF women performed CMR, median age 36 (range 21-67) years. Fifteen were nulliparous. T1 mapping was compared to 30 controls, (14 women) median age 42 (24-64) years. In the left ventricle (LV), T1 times and ECV in all rToF women vs female controls were 1248 ± 61 ms/ 25.8 ± 2.9% vs 1255 ± 40 ms/ 26.8 ± 3.1%, p = 0.7 and p = 0.3, respectively. In rToF, RV T1 times was 1385 ± 124 ms and ECV 37.7 ± 5.4%. There was no association to parity or age in rToF LV T1/ ECV, p = 0.9 for both, or RV T1/ECV, p = 0.4 and p = 0.6, respectively. Indexed LV mass was higher in the rToF pregnancy group, 43 ± 10 vs 38 ± 6 g/m2, p = 0.03 while RV ejection fraction was lower, 49 ± 7% vs 53 ± 6%, p = 0.04. CONCLUSION: Women with rTOF showed evidence of increased RV CMR markers suggestive of diffuse fibrosis while LV CMR markers were within normal values. Having experienced pregnancy might affect RV function, however without association to CMR biomarkers.

Impact of pregnancy and risk factors for ventricular arrhythmias in women with tetralogy of Fallot.

OBJECTIVE: Patients with tetralogy of Fallot (TOF) have high survival rates 30 years after surgical repair. Many patients experience pregnancy; however, the effects of pregnancy on the long-term cardiovascular outcome are not well known. We investigated the association of pregnancy and cardiac function with occurrence of ventricular arrhythmia (VA) in women with TOF. METHODS: We recruited 80 women with repaired TOF from the national database. Holter monitoring or implanted devices detected VA, defined as non-sustained or sustained ventricular tachycardia or aborted cardiac arrest. All patients underwent echocardiography. Blood tests included NT-proBNP (N-terminal pro-brain natriuretic peptide). RESULTS: 55 (69%) women had experienced pregnancy. Mean age was lower in nulliparous compared with those with children (30±9 vs 40±9, p<0.01).VA had occurred in 17 (21%) women. Prevalence of VA was higher in women who had experienced pregnancy (n=16, 94%) compared with nulliparous (n=1, 6%) (p=0.02), also when adjusted for age (OR 12.9 (95% CI 1.5 to 113.2), p=0.02).Right ventricular mechanical dispersion was more pronounced in patients with VA (50±8 ms vs 39±14 ms, p=0.01, age-adjusted OR 2.1 (95% CI 1.3 to 7.5), p=0.01). NT-proBNP was also a marker of VA (211 ng/L (127 to 836) vs 139 ng/L (30 to 465), p=0.007). NT-proBNP >321 ng/L (normal values <170 ng/L) detected women with VA (p=0.019), also independent of age (OR 7.2 (95% CI 1.7 to 30.1), p=0.007). CONCLUSION: Pregnancy was associated with higher prevalence of VA among women with TOF. Right ventricular mechanical dispersion and NT-proBNP were age-independent markers of VA. These may have importance for pregnancy counselling and risk stratification.

An 8-year population description from a national treatment centre on lymphatic malformations.

INTRODUCTION: Lymphatic malformations are rare developmental non-malignant abnormalities that present as masses consisting of abnormal growth of fluid filled channels or spaces in the skin or deep tissue that result in abnormalities of lymphatic flow. They may cause symptoms like pain, recurrent lymphorrea, abnormal bone growth, and disturbance of bodily functions. They may also be a cosmetic problem. The study aims to describe the population with lymphatic malformations (LM) treated at a national referral centre. MATERIALS AND METHODS: This is a retrospective review of patients with the diagnosis lymphatic malformation seen at the centre between 2006-2013. The authors performed a systematic review of the hospital records and a reevaluation of the radiological findings. Eighty-five patients with 102 lymphatic malformations were included. RESULTS: The majority of the patients (67%) were diagnosed before the age of 2 years. Lesions were located mainly to the head and neck (64%) and half (52%) were macrocystic. Treatment was: sclerotherapy (69%), surgery (49%), laser therapy (19%), or a combination of these treatment modalities (33%). The best outcome was found in the patients with macrocystic lesions (p = .029), and 76% of the patients reported a good or very good outcome. CONCLUSION: Most patients presented before the age of 2 years with complex lesions which required unique, often multimodal treatment plans and in general they experienced good outcome.

Pulmonary valve replacement with a bovine pericardial valve: a five year follow-up study.

OBJECTIVES: From a population of 90 patients after pulmonary valve replacement with a biological valve (Carpentier-Edwards Perimount valve), 56 of 80 available patients were examined five years after surgery. BACKGROUND: Pulmonary valve replacement is needed in many patients with congenital heart disease. Homografts have limited availability and predictable degeneration, and mechanical valves require anticoagulation. No superiority of one kind of pulmonary valve replacement has been shown. Biological valves that are readily available are being used and evaluated in increasing numbers. METHODS: In this cross-sectional study, five years following surgery, data were gathered from hospital charts, echocardiography, stress echocardiography, magnetic resonance imaging, and exercise testing. RESULTS: In 90 patients, there were three new valve replacements, one early cardiac death, and four late noncardiac deaths. Echocardiographic assessment of the study group showed pulmonary Doppler velocities (m/s) before, after operation, and at five-year follow-up of 2.8±1.1, 1.6±0.4, and 2.3±0.7, respectively. The assessed insufficiencies (0-3) at the same times were 2.3±1.0, 0.3±0.4, and 1.1±0.8. Maximal oxygen uptake increased from 65.6%±10.1% to 77.1%±18.2% of predicted and QRS width increased by 7±23 ms. Valve degeneration could be associated with young age but not with diagnosis or valve size. CONCLUSION: In our study, the biological valve in the pulmonary position showed excellent mid-term results with few reoperations, low gradients, and mild to moderate insufficiency. Oversizing, in contrast to young age, was not a risk factor for valve degeneration. In younger patients, this allows later percutaneous replacement, reducing the need for further surgery. However, longer follow-up is needed.

Right ventricular volumes assessed by echocardiographic three-dimensional knowledge-based reconstruction compared with magnetic resonance imaging in a clinical setting.

OBJECTIVES: A technique that uses two-dimensional images to create a knowledge-based, three-dimensional model was tested and compared to magnetic resonance imaging. BACKGROUND: Measurement of right ventricular volumes and function is important in the follow-up of patients after pulmonary valve replacement. Magnetic resonance imaging is the gold standard for volumetric assessment. Echocardiographic methods have been validated and are attractive alternatives. METHODS: Thirty patients with tetralogy of Fallot (25 ± 14 years) after pulmonary valve replacement were examined. Magnetic resonance imaging volumetric measurements and echocardiography-based three-dimensional reconstruction were performed. End-diastolic volume, end-systolic volume, and ejection fraction were measured, and the results were compared. RESULTS: Magnetic resonance imaging measurements gave coefficient of variation in the intraobserver study of 3.5, 4.6, and 5.3 and in the interobserver study of 3.6, 5.9, and 6.7 for end-diastolic volume, end-systolic volume, and ejection fraction, respectively. Echocardiographic three-dimensional reconstruction was highly feasible (97%). In the intraobserver study, the corresponding values were 6.0, 7.0, and 8.9 and in the interobserver study 7.4, 10.8, and 13.4. In comparison of the methods, correlations with magnetic resonance imaging were r = 0.91, 0.91, and 0.38, and the corresponding coefficient of variations were 9.4, 10.8, and 14.7. Echocardiography derived volumes (mL/m(2)) were significantly higher than magnetic resonance imaging volumes in end-diastolic volume 13.7 ± 25.6 and in end-systolic volume 9.1 ± 17.0 (both P < .05). CONCLUSIONS: The knowledge-based three-dimensional right ventricular volume method was highly feasible. Intra and interobserver variabilities were satisfactory. Agreement with magnetic resonance imaging measurements for volumes was reasonable but unsatisfactory for ejection fraction. Knowledge-based reconstruction may replace magnetic resonance imaging measurements for serial follow-up, whereas magnetic resonance imaging should be used for surgical decision making.

Radiographic follow-up of community-acquired pneumonia in children.

AIM: To evaluate the value of radiographic follow-up of community-acquired pneumonia in children who are previously healthy. METHODS: Patient records for the years 2003 and 2004 at the Ullevål University Hospital in Oslo were reviewed, and a total of 245 children were selected for the study. Radiographs were evaluated by two paediatric radiologists independently. RESULTS: One hundred and thirty-three patients had control radiographs, of which 106 were normal and 27 were abnormal. Only three of 27 patients with abnormal findings had further clinical problems that could be related to the pneumonia. Two of 106 with normal findings had further clinical problems, despite the normal control radiograph. Of the 112 without radiographic follow-up, 10 had subsequent clinical problems, but most occurred within the first 4 weeks after discharge, before controls would have been scheduled. There were five patients who may have benefited from controls. One relapse could theoretically have been prevented. Four patients were cases for whom the pneumonias were the first manifestations of chronic lung disease. Such patients may have some benefit from control radiographs, but only in terms of detecting the chronic disease at an earlier stage, not in altering the clinical course. Such modest benefits must be weighed against the consequences of providing follow-up to a large number of healthy children, and making lots of abnormal findings with no clinical significance. CONCLUSION: Control radiographs are not very valuable in children who are otherwise healthy.

A prospective randomized comparison between two MRI studies of the small bowel in Crohn's disease, the oral contrast method and MR enteroclysis.

The aim was to compare bowel distension and diagnostic properties of magnetic resonance imaging of the small bowel with oral contrast (MRI per OS) with magnetic resonance enteroclysis (MRE). Forty patients with suspected Crohn's disease (CD) were examined with both MRI methods. MRI per OS was performed with a 6% mannitol solution and MRE with nasojejunal intubation and a polyethylenglycol solution. MRI protocol consisted of balanced fast field echo (B-FFE), T2 and T1 sequences with and without gadolinium. Two experienced radiologists individually evaluated bowel distension and pathological findings including wall thickness (BWT), contrast enhancement (BWE), ulcer (BWU), stenosis (BWS) and edema (EDM). The diameter of the small bowel was smaller with MRI per OS than with MRE (difference jejunum: 0.55 cm, p < 0.001; ileum: 0.35 cm, p < 0.001, terminal ileum: 0.09 cm, p = 0.08). However, CD was diagnosed with high diagnostic accuracy (sensitivity, specificity, positive and negative predictive values: MRI per OS 88%, 89%, 89%, 89%; MRE 88%, 84%, 82%, 89%) and inter-observer agreement (MRI per OS k = 0.95; MRE k = 1). In conclusion, bowel distension was inferior in MRI per OS compared to MRE. However, both methods diagnosed CD with a high diagnostic accuracy and reproducibility.