scholarly journals Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis

2021 ◽  
Vol 9 ◽  
Author(s):  
Fanyan Luo ◽  
Haisong Bu

An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist with associated cardiac lesions, the conduct of the cardiac surgical repair must accommodate the abnormal venous anatomy, especially in total cavopulmonary connection patients. Herein, we present a rare case of a 7-year-old Chinese boy about 1 year post bilateral superior vena cava pulmonary anastomosis who presented with severe cyanosis and was referred to our department. However, the patient showed an unexpected gradual decrease in blood oxygen saturation to 60–70% after the extracardiac total cavopulmonary connection (ETCPC) operation. Emergency echocardiography and computed tomography confirmed that the LHV entered the right atrium. Subsequently, the patient undergone completion of a staged TCPC with intra-atrial tunnel technique. This illustrative report highlights the essence of improving the preoperative accurate diagnosis to avoid unplanned reoperation in China, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Failure to identify anomalous LHV connection, in this case, will delay effective treatment past the optimal treatment time.

Author(s):  
Reza H. Khiabani ◽  
Sulisay Phonekeo ◽  
Harish Srinimukesh ◽  
Elaine Tang ◽  
Mark Fogel ◽  
...  

Single Ventricle Heart Defects (SVHD) are present in 2 per 1000 live births in the US. SVHD are characterized by cyanotic mixing between the de-oxygenated blood from the systemic circulation return and the oxygenated blood from the pulmonary arteries. In the current practice, surgical interventions on SVHD patients commonly result in the total cavopulmonary connection (TCPC) [1]. In this configuration the systemic venous returns (inferior vena cava, IVC, and superior vena cava, SVC) are directly routed to the right and left pulmonary arteries (RPA and LPA), bypassing the right heart. The resulting anatomy has complex and unsteady hemodynamics characterized by flow mixing and flow separation. Pulsation of the inlet venous flow during a cardiac cycle and wall motion may result in complex and unsteady flow patterns in the TCPC. Although vessel wall motion and different degrees of pulsatility have been observed in vivo, non-pulsatile (time-averaged) flow boundary conditions and rigid walls have traditionally been assumed in estimating the TCPC hemodynamic parameters (such as energy loss). Recent studies have shown that these assumptions may result in significant inaccuracies in modeling TCPC hemodynamics [2, 3].


Author(s):  
Elaine Tang ◽  
Doff B. McElhinney ◽  
Ajit P. Yoganathan

2 per 1000 children in the US are born with functionally single ventricle (SV) heart defects. To restore the separate systemic and pulmonary circulations, a Total Cavopulmonary Connection (TCPC) is carried out through a series of surgical steps, which result in the direct connection of the superior vena cava (SVC) and inferior vena cava (IVC) to the pulmonary arteries without an intervening pulmonary ventricle. One way to complete the TCPC is by placing a synthetic patch in the right atrium, forming an intracardiac lateral tunnel (LT) as the final step. As patients grow, some LT pathways become stenosed. The stenosis can impose extra resistance to flow in addition to the TCPC in the SV circulation. One method of treating LT stenosis is by placement of an intravascular stent.


Author(s):  
Maria Restrepo ◽  
Lucia Mirabella ◽  
Elaine Tang ◽  
Chris Haggerty ◽  
Mark A. Fogel ◽  
...  

Single ventricle heart defects affect 2 per 1000 live births in the US and are lethal if left untreated. The Fontan procedure used to treat these defects consists of a series of palliative surgeries to create the total cavopulmonary connection (TCPC), which bypasses the right heart. In the last stage of this procedure, the inferior vena cava (IVC) is connected to the pulmonary arteries (PA) using one of the two approaches: the extra-cardiac (EC), where a synthetic graft is used as the conduit; and the lateral tunnel (LT) where part of the atrial wall is used along with a synthetic patch to create the conduit. The LT conduit is thought to grow in size in the long term because it is formed partially with biological tissue, as opposed to the EC conduit that retains its original size because it contains only synthetic material. The growth of the LT has not been yet quantified, especially in respect to the growth of other vessels forming the TCPC. Furthermore, the effect of this growth on the hemodynamics has not been elucidated. The objective of this study is to quantify the TCPC vessels growth in LT patients from serial magnetic resonance (MR) images, and to understand its effect on the connection hemodynamics using computational fluid dynamics (CFD).


2005 ◽  
Vol 94 (7) ◽  
pp. 469-473 ◽  
Author(s):  
M. Girisch ◽  
L. Sieverding ◽  
R. Rauch ◽  
R. Kaulitz ◽  
M. Gass ◽  
...  

2021 ◽  
pp. 152660282198933
Author(s):  
Pablo V. Uceda ◽  
Julio Peralta Rodriguez ◽  
Hernán Vela ◽  
Adelina Lozano Miranda ◽  
Luis Vega Salvatierra ◽  
...  

The health care system in Peru treats 15,000 dialysis patients annually. Approximately 45% of patients receive therapy using catheters. The incidence of catheter-induced superior vena cava (SVC) occlusion is increasing along with its associated significant morbidity and vascular access dysfunction. One of the unusual manifestations of this complication is bleeding “downhill” esophageal varices caused by reversal of blood flow through esophageal veins around the obstruction to the right atrium. Herein is presented the case of an 18-year-old woman on hemodialysis complicated by SVC occlusion and bleeding esophageal varices who underwent successful endovascular recanalization of the SVC. Bleeding from “downhill” esophageal varices should be considered in the differential diagnosis of dialysis patients exposed to central venous catheters. Aggressive endovascular treatment of SVC occlusion is recommended to preserve upper extremity access function and prevent bleeding from this complication.


Author(s):  
Reina Tonegawa-Kuji ◽  
Kenichiro Yamagata ◽  
Kengo Kusano

Abstract Background  Cough-induced atrial tachycardia (AT) is extremely rare and its electrical origin remains largely unknown. Atrial tachycardias triggered by pharyngeal stimulation, such as swallowing or speech, appears to be more common and the majority of them originate from the superior vena cava or right superior pulmonary vein (PV). Only one case of swallow-triggered AT with right inferior pulmonary vein (RIPV) origin has been reported to date. Case summary  We present a case of a 41-year-old man with recurring episodes of AT in the daytime. He underwent electrophysiology study without sedation. Atrial tachycardia was not observed when the patient entered the examination room and could not be induced with conventional induction procedures. By having the patient cough periodically on purpose, transient AT with P-wave morphology similar to the clinical AT was consistently induced. Activation mapping of the AT revealed a centrifugal pattern with the earliest activity localized inside the RIPV. After successful radiofrequency isolation of the right PV, AT was no longer inducible. Discussion  In the rare case of cough-induced AT originating from the RIPV, the proximity of the inferior right ganglionated plexi (GP) suggests the role of GP in triggering tachycardia. This is the first report that demonstrates voluntary cough was used to induce AT. In such cases that induction of AT is difficult using conventional methods, having the patient cough may be an effective induction method that is easy to attempt.


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