A modified suprasternal approach in transthoracic echocardiography for isolated left upper partial anomalous pulmonary venous connection: Hard to diagnose, easy to miss.

BACKGROUND: Isolated left upper partial anomalous pulmonary venous connection (PAPVC) via the innominate vein to the right atrium is a rare congenital anomaly. This study was undertaken to determine the efficacy of a modified suprasternal view in transthoracic echocardiography (TTE) for the detection of left upper PAPVC. METHODS: After the incidental diagnosis of left upper PAPVC in our first patient in 2008, we added a modified suprasternal view to all TTEs performed in our pediatric cardiology clinic. This was obtained by tilting the tail of the probe 30-450 towards the right shoulder of the patient during suprasternal long axis view for a better visualization of the innominate vein. RESULTS: Among 7200 patients who underwent TTEs between 2008 and 2020, we identified 13 patients with left upper PAPVC into the innominate vein. All were asymptomatic children with normal cardiac chambers and no accompanying congenital disorders. In 10 cases, diagnoses were confirmed by multi-slice computerized tomography, whereas one patient underwent catheterization for confirmation. CONCLUSION: Isolated left upper PAPVC to the innominate vein is a rare congenital disorder that can be present in asymptomatic children with normal cardiac chambers. TTE, with a modified approach in suprasternal long axis view, has a high diagnostic value in the detection of this condition.

Anomalous origin of the left subclavian artery from the pulmonary artery.

Isolated left subclavian artery from the pulmonary trunk is a rare congenital cardiovascular malformation. In this report, we present the images of ascending aortic aneurysm and left subclavian artery originating from the pulmonary artery in a 4-year-old girl in addition to her congenital cardiac pathology.

Surgical treatment of common arterial trunk in patients beyond the first year of life.

BACKGROUND: Common arterial trunk (persistent truncus arteriosus) is a rare cardiac defect requiring surgical repair early in life because of the fast development of pulmonary vascular obstructive disease. We present our institutional experience with patients having common arterial trunk who are diagnosed after one year of age. PATIENTS AND METHODS: Between August 2010 and May 2013, a total of 1,436 patients were treated for congenital cardiac defects at our institution. Common arterial trunk was treated surgically in seven patients older than one year of age (three males, four females; age: 13 months to 5 years, mean: 2.8 ± 2.04 years). All patients underwent cardiac catheterization in order to determine operability. RESULTS: All patients had the aortic dominant type of common arterial trunk. The pulmonary vascular resistance and Qp/Qs ratio before and after oxygen inhalation were mean 9.04 + 4.2 (range: 3.8 and 10.7) wood units and 4.67 ± 2.3 (range: 3 and 6.5) wood units and 3.3 + 1.8 (range: 1.42 and 5.3) and 4.98 + 2.2 (range: 4 and 6.2), respectively. All patients underwent elective primary repair. The ventricular septal defect was closed in all patients, five with a nonvalved patch and two with a unidirectional check-valved patch. Early postoperatively, patients were sedated, hyperventilated, and received nitric oxide for a minimum of 24 hours. There was no early or late mortality. The mean length of hospital stay was 9.3 ± 5.7 days, and mean duration of follow-up was 214 ± 59 days. CONCLUSION: Complete repair of common arterial trunk in patients older than one year of age is feasible in appropriately selected cases. Preoperative cardiac catheterization to assess reactivity of the pulmonary vascular bed is important as are appropriate strategies for postoperative management. Together, these elements make it possible to achieve primary repair with excellent outcomes despite late presentation.

Tetralogy of Fallot with unilateral absent pulmonary artery.

BACKGROUND: Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity. PATIENTS AND METHODS: There were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy. RESULTS: Urgent modified Blalock-Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively. CONCLUSION: Our modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.

Venous and intrapericardial thrombosis: secondary to transient protein C deficiency.

We present a 2-month-old male infant with thrombosis in the superior vena cava and pericardium due to transient protein C deficiency. Protein C deficiency was related to sepsis and hepatitis-induced liver function impairment. The patient's cardiac anatomy was otherwise normal. The patient was referred to us with signs of superior vena cava syndrome. Pericardial mass was excised. Pathological examination diagnosed the mass as organized thrombus. After the operation, signs of superior vena cava syndrome totally resolved. Serial echocardiographic examinations revealed regression of thrombus in the superior vena cava. This is the first case reported in the literature with intrapericardial thrombus secondary to transient protein C deficiency.

[Fetal arrhythmias: diagnosis, treatment and prognosis]. / Fetal Aritmiler: Tani, Tedavi, Prognoz.

OBJECTIVE: To evaluate patients who were referred to our center with suspicion of fetal arrhythmia for diagnosis, results of treatment and prognosis. METHODS: Thirty-three patients referred to our center for evaluation of fetal cardiac arrhythmias were examined by M-mode, two-dimensional and Doppler echocardiography for cardiac anomaly and type of dysrhythmia RESULTS: Arrhythmias were diagnosed in 15 of 33 patients. Four cases demonstrated tachycardia, 5--ectopic beats, 6--bradycardia. Of 5 fetuses with tachycardia, transplacental antiarrhythmic drugs were administered in three cases; two of them with hydrops died and conversion of the arrhythmia was achieved in one. Six patients with bradycardia had poor perinatal outcome, with 3 deaths occurred (two of them had third degree atrioventricular block, one of them had sinus bradycardia). All patients with ectopic beats had a good perinatal outcome. CONCLUSION: The results suggest that bradycardic and/or hydropic fetuses have poor prognosis, however fetuses with ectopic beats generally have desirable prognosis.