A rare cause of platypnea-orthodeoxia syndrome in a young female due to persistent left superior vena cava.

Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath in the upright position that is relieved with supine positioning. We present a rare case of persistent left superior vena cava (PLSVC) draining into the left atrium causing a right-to-left shunt and subsequent POS. A 30-year-old female with a past medical history of hypertrophic cardiomyopathy, congenital Long QT syndrome and a left-sided dual chamber implantable cardioverter-defibrillator (ICD) presented with dyspnea and lightheadedness. Prior to presentation, the patient underwent a left-sided ICD extraction due to ICD lead infection and re-implantation from the right side through the cephalic vein. After further investigation, it was concluded that the PLSVC resulted in a physiological right-to-left shunting causing POS, with resolution of her symptoms after surgical ligation. To our knowledge, this is the first case report of PLSVC presenting with POS without anatomical intracardiac shunts following iatrogenic right superior vena cava (RSVC) obstruction.

Surgical Timing and Outcomes of Unilateral Versus Bilateral Superior Cavopulmonary Anastomosis: An Analysis of Pediatric Heart Network Public Databases.

Requiring bilateral superior cavopulmonary anastomosis (bSCPA) instead of unilateral superior cavopulmonary anastomosis (uSCPA) could influence surgical timing and outcomes. We compared surgical timing and outcomes for patients who underwent uSCPA to those who underwent bSCPA through use of the Pediatric Heart Network's public datasets for the Infant Single Ventricle trial and Single Ventricle Reconstruction trial. There was no statistically significant difference in median age at SCPA (158 vs. 150 days, p = 0.68), hospital length of stay (LOS) (7 vs. 7 days, p = 0.74), intensive care unit (ICU) LOS (4 vs. 5 days, p = 0.53), time requiring ventilator support (2 vs. 2 days, p = 0.51), or oxygen saturation at discharge (82 vs. 81%, p = 0.22) between the uSCPA and bSCPA groups, respectively. However, sub-analysis comparing only those who underwent early SCPA, at < 120 days of age, revealed significantly longer hospital LOS (8 vs. 13 days, p = 0.04), ICU LOS (5 vs. 11 days, p = 0.01), and time requiring ventilator support (2 vs. 4 days, p = 0.03) for the early bSCPA group when compared to the early uSCPA group. A multivariable logistic regression revealed bSCPA to be the only significant predictor of prolonged hospital LOS for patients who underwent early SCPA (odds ratio 4.1, 95% CI 1.2-14.2). Overall, there was no difference in surgical timing or outcome measures between uSCPA and bSCPA. However, early bSCPA, performed at < 120 days, had worse outcome measures than early uSCPA. Delaying elective bSCPA until at least 120 days of age could minimize morbidity in infants with bilateral superior venae cavae.

Impact of persistent left superior vena cava on radiofrequency catheter ablation in patients with atrial fibrillation.

AIMS: The impact of persistent left superior vena cava (PLSVC) in atrial fibrillation (AF) patients undergoing radiofrequency catheter ablation (RFCA) is not well known. We performed this analysis to evaluate the electrophysiological characteristics of PLSVC and its role in triggering and maintaining AF. METHODS AND RESULTS: Patients with AF referred to two tertiary hospitals were screened and patients with PLSVC in pre-RFCA imaging studies were enrolled. Among 3967 patients, PLSVC was present in 36 patients (0.9%). There were four morphological types of PLSVC: type 1, atresia of the right superior vena cava (SVC) (n = 2); type 2A, dual SVCs with an anastomosis between right and left SVCs (n = 15); type 2B, dual SVCs without an anastomosis (n = 16); type 3, PLSVC draining into the left atrium (LA; n = 2); and unclassified in one patient. Thirty-two patients underwent RFCA and electrophysiology study focusing on PLSVC: PLSVC was the trigger of AF in 48.4% of patients and the driver of AF in 46.9% of patients. Cumulatively, PLSVC was a trigger or driver of AF in 22 patients (68.8%). Whether to ablate PLSVC was determined by the results of electrophysiology study, and no significant difference in the late recurrence rate was observed between patients who did and did not have either trigger or driver from PLSVC. CONCLUSION: Pre-RFCA cardiac imaging revealed PLSVC in 0.9% of AF patients. This study demonstrated that PLSVC has an important role in initiating and maintaining AF in substantial proportion of patients. Electrophysiology study focusing on PLSVC can help to decide whether to ablate PLSVC.

Challenge Accepted: Lead Extraction in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Occlusion.

Persistent left superior vena cava (PLSVC) is an anatomic variant that is relatively uncommon in the general population. Lead extraction through PLSVC is extremely rare. Due to unusual anatomy, the procedure carries challenges that require special considerations and careful planning. The authors report a case of lead extraction through a PLSVC with occluded right superior vena cava and highlight the challenges and outcomes of the procedure.

Persistent Left Superior Vena Cava in a Heart Transplant Recipient's Native Heart: A Rare Case Diagnosed by Transesophageal Echocardiogram.

Persistent left superior vena cava (PLSVC) is a rare abnormality with incidence reported as 0.3% to 0.5% in the general population and about 10 times higher in patients with congenital heart disease. The diagnosis of PLSVC in native or donor hearts of patients undergoing heart transplants has been reported in surgical journals. However, this rare finding has not been described in similar heart transplant settings in anesthesia literature. This case describes a 44-year-old male orthotopic heart transplant recipient who was incidentally diagnosed with PLSVC in his native heart on transesophageal echocardiogram after a central venous catheter placement. The particular position of the central venous catheter, in our case, raised the suspicion of PLSVC but needed further verification. With the help of images and videos, we demonstrate that transesophageal echocardiogram can be instrumental in diagnosing PLSVC. Furthermore, the case highlights the importance of effectively communicating with the surgeon about such a finding so that the surgical plan can be modified in a timely manner.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Left upper lung cancer with persistent left superior vena cava and left azygos vein: a case report.

BACKGROUND: With the popularization of thoracoscopic surgery, more and more macrovascular malformations have been reported. Understanding some vascular malformations with relatively fixed anatomical site and their range of drainage could avoid severe complications during the surgery. Persistent left superior vena cava (PLSVC) is a common thoracic vascular malformation, and is always combined with other cardiovascular dysplasia. As for the patient with upper left lung cancer in this case, he had PLSVC and left azygos vein, and non-metastatic enlargement of the lymph nodes at the same time, which had influenced the decisions on surgery and treatment. We made a summary of experience regarding this. CASE PRESENTATION: A 46-years-old male patient, his CT found a space-occupying lesion in the superior lobe of the left lung. The chest CT showed that the patient had PLSVC and left azygos vein, and multiple enlarged lymph nodes in the mediastinum. The patient received thoracoscopic upper left lung lobectomy and lymph node dissection. It was discovered that the left azygos vein had a concealed form, which influenced the lymph node dissection. The post-surgery pathology showed that there was squamous cell carcinoma in the upper left lung (pT2bN0M0 p Phase IIA) and no cancer metastasis with the lymph nodes. The patient had a good post-surgery recovery. CONCLUSIONS: PLSVC is not rare, and is always combined with other vascular malformations. If discovering PLSVC before surgery, we suggest completing chest enhanced CT and vascular reconstruction, to find out other cardiovascular malformations that may exist. Left azygos vein is a rare vascular malformation, but it has a relatively fixed anatomical site, and always co-exists with PLSVC, therefore, understanding anatomy of left azygos vein is good for preventing accidental damage. Especially when performing surgery above the left pulmonary artery trunk, attention shall be paid to preventing damage to the left azygos vein. In addition, as for the patient with the diagnosis of lung cancer before surgery, it is not reliable to judge whether there is metastasis or not merely according to the size of the lymph nodes, instead, PET-CT or needle biopsy is recommended.